"Medicine, Faculty of"@en . "Medical Genetics, Department of"@en . "DSpace"@en . "UBCV"@en . "Fisher, Emily Rachel"@en . "2012-10-02T16:37:58Z"@en . "2012"@en . "Master of Science - MSc"@en . "University of British Columbia"@en . "Introduction: Global prevalence estimates for Huntington Disease (HD) vary widely, and those cited for Canada are outdated and not specific to British Columbia (BC). The most recent incidence calculation was performed in BC and includes diagnoses only up to the year 1999. Reports on the population at risk in Canada are based on theories and estimates that do not pertain to any particular population. Despite the presence of an extensive laboratory and clinical research hub in this province, a comprehensive epidemiological study of the prevalence, incidence and population at risk for HD has never been assessed. As such, the specific objectives of this study were to: 1) Calculate the minimum prevalence of HD in BC on April 1, 2012; 2) Calculate the incidence of HD in BC from January 1, 2001- December 31, 2011; and 3) Calculate the minimum population at risk for HD in BC on April 1, 2012. \r\nMethods: A comprehensive province-wide assessment of the HD patient population and the population at risk was conducted using multiple sources of ascertainment including: UBC HD clinic records, hospital and physician records, DNA diagnostic lab reports, the HD research lab at the Centre for Molecular Medicine and Therapeutics (CMMT), nursing homes, The Huntington Society of Canada and HD community members. \r\nResults: The minimum prevalence of HD in BC was estimated at 12.5 - 14.9/100,000 (95% CI: 11.5-16.0) (1/8,697 \u00E2\u0080\u0093 1/6,250), the incidence, 7.2 per million/year (95% CI: 6.5-7.9), and the minimum population at risk: 1/1,064 (95% CI: 1/1,941 - 1/2,107).\r\nConclusions: The prevalence of HD is nearly twice as high as suggested by a previous Canadian report. This study comprised the most thorough HD patient ascertainment study since the advent of direct mutation testing and may set a precedent for future prevalence studies. Incidence has remained the same since 1999 and BC is only the fourth region in the world to provide a direct estimate of the population at risk for HD."@en . "https://circle.library.ubc.ca/rest/handle/2429/43319?expand=metadata"@en . "\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 THE\t\r \u00C2\u00A0EPIDEMIOLOGY\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HUNTINGTON\t\r \u00C2\u00A0DISEASE\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BRITISH\t\r \u00C2\u00A0COLUMBIA\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 by\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Emily\t\r \u00C2\u00A0Rachel\t\r \u00C2\u00A0Fisher\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0BSc,\t\r \u00C2\u00A0The\t\r \u00C2\u00A0University\t\r \u00C2\u00A0of\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia,\t\r \u00C2\u00A02012\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 08\t\r \u00C2\u00A0Fall\t\r \u00C2\u00A0 THESIS\t\r \u00C2\u00A0SUBMITTED\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0PARTIAL\t\r \u00C2\u00A0FUFILLMENT\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0REQUIREMENTS\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0DEGREE\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0\t\r \u00C2\u00A0MASTER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0SCIENCE\t\r \u00C2\u00A0\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Faculty\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Graduate\t\r \u00C2\u00A0Studies\t\r \u00C2\u00A0\t\r \u00C2\u00A0(Medical\t\r \u00C2\u00A0Genetics)\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0UNIVERSITY\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0BRITISH\t\r \u00C2\u00A0COLUMBIA\t\r \u00C2\u00A0(Vancouver)\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0September\t\r \u00C2\u00A02012\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\u00C2\u00A9\t\r \u00C2\u00A0Emily\t\r \u00C2\u00A0Rachel\t\r \u00C2\u00A0Fisher,\t\r \u00C2\u00A02012\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 ii\t\r \u00C2\u00A0 Abstract\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Introduction:\t\r \u00C2\u00A0Global\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0(HD)\t\r \u00C2\u00A0vary\t\r \u00C2\u00A0widely,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0those\t\r \u00C2\u00A0cited\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0are\t\r \u00C2\u00A0outdated\t\r \u00C2\u00A0and\t\r \u00C2\u00A0not\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0to\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0(BC).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0was\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0and\t\r \u00C2\u00A0includes\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0only\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A01999.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Reports\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0are\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0theories\t\r \u00C2\u00A0and\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0that\t\r \u00C2\u00A0do\t\r \u00C2\u00A0not\t\r \u00C2\u00A0pertain\t\r \u00C2\u00A0to\t\r \u00C2\u00A0any\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Despite\t\r \u00C2\u00A0the\t\r \u00C2\u00A0presence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0an\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0laboratory\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0research\t\r \u00C2\u00A0hub\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0comprehensive\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0study\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0has\t\r \u00C2\u00A0never\t\r \u00C2\u00A0been\t\r \u00C2\u00A0assessed.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0such,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0objectives\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0were\t\r \u00C2\u00A0to:\t\r \u00C2\u00A01)\t\r \u00C2\u00A0Calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0on\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012;\t\r \u00C2\u00A02)\t\r \u00C2\u00A0Calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0from\t\r \u00C2\u00A0January\t\r \u00C2\u00A01,\t\r \u00C2\u00A02001-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0December\t\r \u00C2\u00A031,\t\r \u00C2\u00A02011;\t\r \u00C2\u00A0and\t\r \u00C2\u00A03)\t\r \u00C2\u00A0Calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0on\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Methods:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0comprehensive\t\r \u00C2\u00A0province-\u00C2\u00AD\u00E2\u0080\u0090wide\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0was\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0using\t\r \u00C2\u00A0multiple\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0including:\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0records,\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0and\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0records,\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0reports,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Molecular\t\r \u00C2\u00A0Medicine\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Therapeutics\t\r \u00C2\u00A0(CMMT),\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes,\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Society\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0and\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0members.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Results:\t\r \u00C2\u00A0The\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0at\t\r \u00C2\u00A012.5\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A014.9/100,000\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A011.5-\u00C2\u00AD\u00E2\u0080\u009016.0)\t\r \u00C2\u00A0(1/8,697\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A01/6,250),\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence,\t\r \u00C2\u00A07.2\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A06.5-\u00C2\u00AD\u00E2\u0080\u00907.9),\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk:\t\r \u00C2\u00A01/1,064\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A01/1,941\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A01/2,107).\t\r \u00C2\u00A0 Conclusions:\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0is\t\r \u00C2\u00A0nearly\t\r \u00C2\u00A0twice\t\r \u00C2\u00A0as\t\r \u00C2\u00A0high\t\r \u00C2\u00A0as\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0report.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0study\t\r \u00C2\u00A0comprised\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0thorough\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0study\t\r \u00C2\u00A0since\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0and\t\r \u00C2\u00A0may\t\r \u00C2\u00A0set\t\r \u00C2\u00A0a\t\r \u00C2\u00A0precedent\t\r \u00C2\u00A0for\t\r \u00C2\u00A0future\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0studies.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0has\t\r \u00C2\u00A0remained\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0since\t\r \u00C2\u00A01999\t\r \u00C2\u00A0and\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0is\t\r \u00C2\u00A0only\t\r \u00C2\u00A0the\t\r \u00C2\u00A0fourth\t\r \u00C2\u00A0region\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0world\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0a\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 iii\t\r \u00C2\u00A0 Preface\t\r \u00C2\u00A0Study\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0were\t\r \u00C2\u00A0approved\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Children's\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Women's\t\r \u00C2\u00A0Research\t\r \u00C2\u00A0Ethics\t\r \u00C2\u00A0Board\t\r \u00C2\u00A0(H10-\u00C2\u00AD\u00E2\u0080\u009000943).\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Cure\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Initiative\t\r \u00C2\u00A0(CHDI)\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0funding\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0project\t\r \u00C2\u00A0(CHDI,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 iv\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Contents\t\r \u00C2\u00A0 Abstract\t\r \u00C2\u00A0.......................................................................................................................................\t\r \u00C2\u00A0ii\t\r \u00C2\u00A0 Preface\t\r \u00C2\u00A0.......................................................................................................................................\t\r \u00C2\u00A0iii\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Contents\t\r \u00C2\u00A0....................................................................................................................\t\r \u00C2\u00A0iv\t\r \u00C2\u00A0 List\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Tables\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A0vi\t\r \u00C2\u00A0 List\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Figures\t\r \u00C2\u00A0.........................................................................................................................\t\r \u00C2\u00A0vii\t\r \u00C2\u00A0 Acknowledgements\t\r \u00C2\u00A0................................................................................................................\t\r \u00C2\u00A0ix\t\r \u00C2\u00A0 Introduction\t\r \u00C2\u00A0..............................................................................................................................\t\r \u00C2\u00A01\t\r \u00C2\u00A0 Background\t\r \u00C2\u00A0........................................................................................................................................\t\r \u00C2\u00A01\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0........................................................................................................................................\t\r \u00C2\u00A01\t\r \u00C2\u00A0Epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0.....................................................................................................\t\r \u00C2\u00A02\t\r \u00C2\u00A0 Rationale\t\r \u00C2\u00A0.....................................................................................................................................\t\r \u00C2\u00A08\t\r \u00C2\u00A0 Specific\t\r \u00C2\u00A0objectives\t\r \u00C2\u00A0................................................................................................................\t\r \u00C2\u00A011\t\r \u00C2\u00A0 Methodology\t\r \u00C2\u00A0...........................................................................................................................\t\r \u00C2\u00A012\t\r \u00C2\u00A0 Epidemiology:\t\r \u00C2\u00A0definitions\t\r \u00C2\u00A0............................................................................................................\t\r \u00C2\u00A012\t\r \u00C2\u00A0 Population\t\r \u00C2\u00A0under\t\r \u00C2\u00A0review:\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0.........................................................................\t\r \u00C2\u00A013\t\r \u00C2\u00A0 Defining\t\r \u00C2\u00A0an\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patient:\t\r \u00C2\u00A0Age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0..............................................................................\t\r \u00C2\u00A013\t\r \u00C2\u00A0 Sources\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Ascertainment\t\r \u00C2\u00A0............................................................................................................\t\r \u00C2\u00A015\t\r \u00C2\u00A0 Methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Ascertainment\t\r \u00C2\u00A0..........................................................................................................\t\r \u00C2\u00A016\t\r \u00C2\u00A0 Maximizing\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0numbers:\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0 scores\t\r \u00C2\u00A0..................................................................................................................................................\t\r \u00C2\u00A021\t\r \u00C2\u00A0Certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0.....................................................................................................................................\t\r \u00C2\u00A021\t\r \u00C2\u00A0Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0..........................................................................................\t\r \u00C2\u00A022\t\r \u00C2\u00A0 Performing\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0...............................................................................................................\t\r \u00C2\u00A027\t\r \u00C2\u00A0Minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0...................................................................................................................\t\r \u00C2\u00A027\t\r \u00C2\u00A0Mortality\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0.....................................................................................................................................\t\r \u00C2\u00A029\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A0ethnicity\t\r \u00C2\u00A0.............................................................................................................................\t\r \u00C2\u00A029\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0..........................................................................................................................................................\t\r \u00C2\u00A031\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0................................................................................................................................\t\r \u00C2\u00A032\t\r \u00C2\u00A0 Results\t\r \u00C2\u00A0......................................................................................................................................\t\r \u00C2\u00A037\t\r \u00C2\u00A0 Patient\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0results\t\r \u00C2\u00A0....................................................................................................\t\r \u00C2\u00A037\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0.......................................................................................\t\r \u00C2\u00A042\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0......................................................................................................\t\r \u00C2\u00A043\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0composition\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population\t\r \u00C2\u00A0.....................................................\t\r \u00C2\u00A044\t\r \u00C2\u00A0Geographic\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0.....................................................................................................\t\r \u00C2\u00A045\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0.......................................................................................................................\t\r \u00C2\u00A047\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0.......................................................................................................\t\r \u00C2\u00A050\t\r \u00C2\u00A0Predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0.........................................................................................................................................\t\r \u00C2\u00A054\t\r \u00C2\u00A0 Discussion\t\r \u00C2\u00A0...............................................................................................................................\t\r \u00C2\u00A056\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0.........................................................................................................................................\t\r \u00C2\u00A056\t\r \u00C2\u00A0 Incidence\t\r \u00C2\u00A0...........................................................................................................................................\t\r \u00C2\u00A067\t\r \u00C2\u00A0 Population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0...........................................................................................................................\t\r \u00C2\u00A071\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 v\t\r \u00C2\u00A0 Predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A074\t\r \u00C2\u00A0 Direct\t\r \u00C2\u00A0and\t\r \u00C2\u00A0immediate\t\r \u00C2\u00A0implications\t\r \u00C2\u00A0................................................................................\t\r \u00C2\u00A077\t\r \u00C2\u00A0 Conclusion\t\r \u00C2\u00A0...............................................................................................................................\t\r \u00C2\u00A079\t\r \u00C2\u00A0 Future\t\r \u00C2\u00A0direction\t\r \u00C2\u00A0..............................................................................................................................\t\r \u00C2\u00A080\t\r \u00C2\u00A0 Bibliography\t\r \u00C2\u00A0...........................................................................................................................\t\r \u00C2\u00A083\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A01a\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A091\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A01b\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A094\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A02\t\r \u00C2\u00A0..............................................................................................................................\t\r \u00C2\u00A097\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A03\t\r \u00C2\u00A0..............................................................................................................................\t\r \u00C2\u00A099\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A04\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A0100\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A05\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A0101\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A06\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A0102\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A07\t\r \u00C2\u00A0............................................................................................................................\t\r \u00C2\u00A0106\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 vi\t\r \u00C2\u00A0 List\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Tables\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A01.\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0PREVALENCE,\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0AT\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0THAT\t\r \u00C2\u00A0TOOK\t\r \u00C2\u00A0PLACE\t\r \u00C2\u00A0AFTER\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0ADVENT\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0DIRECT\t\r \u00C2\u00A0MUTATION\t\r \u00C2\u00A0TEST\t\r \u00C2\u00A0(POST\t\r \u00C2\u00A01993)\t\r \u00C2\u00A0..................................................................................................\t\r \u00C2\u00A03\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A02.\t\r \u00C2\u00A0FIELDS\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0ACCESS\t\r \u00C2\u00A0DATABASE\t\r \u00C2\u00A0PERTAINING\t\r \u00C2\u00A0TO\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0CALCULATIONS\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PREVALENCE,\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0AT\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0...................................................................................................................................................\t\r \u00C2\u00A017\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A03.\t\r \u00C2\u00A0NUMBERS\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0PROPORTION\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0PATIENTS\t\r \u00C2\u00A0DIAGNOSED\t\r \u00C2\u00A0WITH\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0WITHOUT\t\r \u00C2\u00A0A\t\r \u00C2\u00A0GENETIC\t\r \u00C2\u00A0TEST\t\r \u00C2\u00A0.................\t\r \u00C2\u00A038\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A04.\t\r \u00C2\u00A0SUMMARY\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PATIENTS\t\r \u00C2\u00A0ASCERTAINED\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A0PHYSICIAN\t\r \u00C2\u00A0QUESTIONNAIRES\t\r \u00C2\u00A0.........................................................\t\r \u00C2\u00A039\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A05.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PATIENTS\t\r \u00C2\u00A0ASCERTAINED\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0SOURCE\t\r \u00C2\u00A0UNDER\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0CERTAINTY\t\r \u00C2\u00A0MEASURE\t\r \u00C2\u00A0OR\t\r \u00C2\u00A0OVERLAP\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0SCORE\t\r \u00C2\u00A0IS\t\r \u00C2\u00A0SHOWN\t\r \u00C2\u00A0BELOW.\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0UPPER,\t\r \u00C2\u00A0MID\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0LOWER\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0RANGE\t\r \u00C2\u00A0CALCULATIONS\t\r \u00C2\u00A0IS\t\r \u00C2\u00A0BROKEN\t\r \u00C2\u00A0DOWN\t\r \u00C2\u00A0......................................................................................................................................\t\r \u00C2\u00A042\t\r \u00C2\u00A0TABLE\t\r \u00C2\u00A06.\t\r \u00C2\u00A0\t\r \u00C2\u00A0BREAKDOWN\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0CALCULATION\t\r \u00C2\u00A0SHOWING\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0CASES\t\r \u00C2\u00A0INCLUDED\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0SOURCE,\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0TOTAL\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0INCIDENT\t\r \u00C2\u00A0CASES\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0DIAGNOSTIC\t\r \u00C2\u00A0TEST\t\r \u00C2\u00A0(DT)-\u00C2\u00AD\u00E2\u0080\u0090POSITIVE\t\r \u00C2\u00A0RATE\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0YEAR\t\r \u00C2\u00A0BETWEEN\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011\t\r \u00C2\u00A0...............................................................................................\t\r \u00C2\u00A048\t\r \u00C2\u00A0TABLE\t\r 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\u00C2\u00A0.......................................................................................................................................................\t\r \u00C2\u00A033\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A011.\t\r \u00C2\u00A0\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0EQUATION\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0CALCULATING\t\r \u00C2\u00A0UPTAKE\t\r \u00C2\u00A0AS\t\r \u00C2\u00A0PROPOSED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0TASSICKER\t\r \u00C2\u00A0ET\t\r \u00C2\u00A0AL.\t\r \u00C2\u00A0(2009)\t\r \u00C2\u00A0............................\t\r \u00C2\u00A034\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A012.\t\r \u00C2\u00A0A\t\r \u00C2\u00A0DEPICTION\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0THEORY\t\r \u00C2\u00A0PROPOSED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0TASSICKER\t\r \u00C2\u00A0ET\t\r \u00C2\u00A0AL.\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0SUPPORT\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PROPOSED\t\r \u00C2\u00A0EQUATION\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0CALCULATING\t\r \u00C2\u00A0UPTAKE\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PREDICTIVE\t\r \u00C2\u00A0TESTING.\t\r \u00C2\u00A0DOTTED\t\r \u00C2\u00A0VERTICAL\t\r \u00C2\u00A0LINES\t\r \u00C2\u00A0ENCLOSE\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0EXAMPLE\t\r \u00C2\u00A0STUDY\t\r \u00C2\u00A0PERIOD.\t\r \u00C2\u00A0AN\t\r \u00C2\u00A0INDIVIDUAL\t\r \u00C2\u00A0AFFECTED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0(HD1)\t\r \u00C2\u00A0IS\t\r \u00C2\u00A0REPLACED\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PATIENT\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0A\t\r \u00C2\u00A0NEWLY\t\r \u00C2\u00A0AFFECTED\t\r \u00C2\u00A0INDIVIDUAL\t\r \u00C2\u00A0(HD2),\t\r \u00C2\u00A0THEREBY\t\r \u00C2\u00A0MAINTAINING\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0OVER\t\r \u00C2\u00A0TIME.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NEWLY\t\r \u00C2\u00A0AFFECTED\t\r \u00C2\u00A0INDIVIDUAL\t\r \u00C2\u00A0NOW\t\r \u00C2\u00A0REPRESENTS\t\r \u00C2\u00A0ADDITIONAL\t\r \u00C2\u00A0MEMBERS\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A050%\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0POPULATION.\t\r \u00C2\u00A0THEORETICALLY,\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THESE\t\r \u00C2\u00A0ADDITIONAL\t\r \u00C2\u00A0INDIVIDUALS\t\r \u00C2\u00A0IS\t\r \u00C2\u00A0EQUAL\t\r \u00C2\u00A0TO\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A050%\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0INDIVIDUALS\t\r \u00C2\u00A0REPRESENTED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PREVIOUSLY\t\r \u00C2\u00A0DECEASED.\t\r \u00C2\u00A0...............................................................................................................\t\r \u00C2\u00A035\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A013.\t\r \u00C2\u00A0VENN\t\r \u00C2\u00A0DIAGRAMS\t\r \u00C2\u00A0SHOWING\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PATIENT\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0BREAKDOWN\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0LOWER,\t\r \u00C2\u00A0B.\t\r \u00C2\u00A0MID\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0UPPER\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0RANGE\t\r \u00C2\u00A0.............................................................................................................................................................\t\r \u00C2\u00A043\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A014.\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0PATIENTS\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0SEPARATED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0ETHNICITY.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PIE\t\r \u00C2\u00A0CHART\t\r \u00C2\u00A0SHOWS\t\r \u00C2\u00A0RELATIVE\t\r \u00C2\u00A0PROPORTIONS\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0ETHNIC\t\r \u00C2\u00A0GROUP\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\u00E2\u0080\u0099S\t\r \u00C2\u00A0PATIENT\t\r \u00C2\u00A0POPULATION.\t\r \u00C2\u00A0BREAKDOWN\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0\u00E2\u0080\u0098OTHER\u00E2\u0080\u0099\t\r \u00C2\u00A0CATEGORY\t\r \u00C2\u00A0SHOWS\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PROPORTION\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0BC\u00E2\u0080\u0099S\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0LISTED\t\r \u00C2\u00A0ETHNIC\t\r \u00C2\u00A0GROUP.\t\r \u00C2\u00A0ETHNIC-\u00C2\u00AD\u00E2\u0080\u0090SPECIFIC\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0ARE\t\r \u00C2\u00A0SHOWN\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0COLUMN\t\r \u00C2\u00A04\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0TABLE.\t\r \u00C2\u00A0...................................................................................................\t\r \u00C2\u00A044\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A015.\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0SEPARATED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0\u00E2\u0080\u009CRURAL\u00E2\u0080\u009D\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0\u00E2\u0080\u009CURBAN\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0.....................................................................\t\r \u00C2\u00A045\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A016.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PATIENTS\t\r \u00C2\u00A0LIVING\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0BRITISH\t\r \u00C2\u00A0COLUMBIAN\t\r \u00C2\u00A0HEALTH\t\r \u00C2\u00A0REGION\t\r \u00C2\u00A0BASED\t\r \u00C2\u00A0ON\t\r \u00C2\u00A0LOWER\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0UPPER\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0RANGE.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0COLOUR\t\r \u00C2\u00A0KEY\t\r \u00C2\u00A0SHOWS\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0RESPECTIVE\t\r \u00C2\u00A0REGION.\t\r \u00C2\u00A0..................................................................................................................................................................................\t\r \u00C2\u00A046\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A017.\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0(/MILLION/YEAR)\t\r \u00C2\u00A0FROM\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011.\t\r \u00C2\u00A0ERROR\t\r \u00C2\u00A0BARS\t\r \u00C2\u00A0REPRESENT\t\r \u00C2\u00A095%\t\r \u00C2\u00A0CONFIDENCE\t\r \u00C2\u00A0INTERVALS\t\r \u00C2\u00A0.....................................................................................................................................................\t\r \u00C2\u00A047\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A018.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0AT\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0SEPARATED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0\u00E2\u0080\u009CRURAL\u00E2\u0080\u009D\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0\u00E2\u0080\u009CURBAN\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0.............................................\t\r \u00C2\u00A051\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A019.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0AT\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0PROVINCIAL\t\r \u00C2\u00A0HEALTH\t\r \u00C2\u00A0REGION\t\r \u00C2\u00A0......................................................\t\r \u00C2\u00A052\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A020.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0PREDICTIVE\t\r \u00C2\u00A0TESTS\t\r \u00C2\u00A0PROVIDED\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0BETWEEN\t\r \u00C2\u00A01987\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0APRIL\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0BOTTOM\t\r \u00C2\u00A0SECTION\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0BAR\t\r \u00C2\u00A0SHOWS\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0LINKAGE\t\r \u00C2\u00A0TESTS\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0TOP\t\r \u00C2\u00A0PORTION\t\r \u00C2\u00A0SHOWS\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0NUMBER\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0DIRECT\t\r \u00C2\u00A0MUTATION\t\r \u00C2\u00A0TESTS\t\r \u00C2\u00A0....................................................................................................................................................\t\r \u00C2\u00A054\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A021.\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0ESTIMATE\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0EACH\t\r \u00C2\u00A0REGION\t\r \u00C2\u00A0SHOWN\t\r \u00C2\u00A0OVER\t\r \u00C2\u00A0TIME\t\r \u00C2\u00A0INCLUDING\t\r \u00C2\u00A0UPDATED\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0AVERAGE\t\r \u00C2\u00A0...........\t\r \u00C2\u00A057\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A022.\t\r \u00C2\u00A0CANADIAN\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0OVER\t\r \u00C2\u00A0TIME.\t\r \u00C2\u00A0ERROR\t\r \u00C2\u00A0BARS\t\r \u00C2\u00A0REPRESENT\t\r \u00C2\u00A095%\t\r \u00C2\u00A0CONFIDENCE\t\r \u00C2\u00A0INTERVALS.\t\r \u00C2\u00A0........................................................................................................................................................................................\t\r \u00C2\u00A057\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A023.\t\r \u00C2\u00A0LIFE\t\r \u00C2\u00A0EXPECTANCY\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0CANADA\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0WORLDWIDE\t\r \u00C2\u00A0(1960-\u00C2\u00AD\u00E2\u0080\u00902011).\t\r \u00C2\u00A0.....................................................................\t\r \u00C2\u00A060\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A024A.\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0SINCE\t\r \u00C2\u00A01987\t\r \u00C2\u00A0USING\t\r \u00C2\u00A0CLINICAL\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0GENETIC\t\r \u00C2\u00A0DIAGNOSTIC\t\r \u00C2\u00A0CRITERIA.\t\r \u00C2\u00A0ERROR\t\r \u00C2\u00A0BARS\t\r \u00C2\u00A0REPRESENT\t\r \u00C2\u00A095%\t\r \u00C2\u00A0CONFIDENCE\t\r \u00C2\u00A0INTERVALS\t\r \u00C2\u00A0..............................................................................................\t\r \u00C2\u00A068\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A024B.\t\r \u00C2\u00A0\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0SINCE\t\r \u00C2\u00A01993\t\r \u00C2\u00A0USING\t\r \u00C2\u00A0GENETIC\t\r \u00C2\u00A0DIAGNOSTIC\t\r \u00C2\u00A0CRITERIA\t\r \u00C2\u00A0ONLY.\t\r \u00C2\u00A0ERROR\t\r \u00C2\u00A0BARS\t\r \u00C2\u00A0REPRESENT\t\r \u00C2\u00A095%\t\r \u00C2\u00A0CONFIDENCE\t\r \u00C2\u00A0INTERVALS\t\r \u00C2\u00A0...........................................................................................................\t\r \u00C2\u00A069\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 viii\t\r \u00C2\u00A0 FIGURE\t\r \u00C2\u00A025.\t\r \u00C2\u00A0GLOBAL\t\r \u00C2\u00A0ESTIMATES\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0INCIDENCE\t\r \u00C2\u00A0OVER\t\r \u00C2\u00A0TIME\t\r \u00C2\u00A0AND\t\r \u00C2\u00A0GEOGRAPHIC\t\r \u00C2\u00A0REGION.\t\r \u00C2\u00A0\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0COMPARATIVE\t\r \u00C2\u00A0PURPOSES,\t\r \u00C2\u00A0STUDIES\t\r \u00C2\u00A0AFTER\t\r \u00C2\u00A01993\t\r \u00C2\u00A0INCLUDE\t\r \u00C2\u00A0ONLY\t\r \u00C2\u00A0DIAGNOSES\t\r \u00C2\u00A0CONFIRMED\t\r \u00C2\u00A0BY\t\r \u00C2\u00A0GENETIC\t\r \u00C2\u00A0TEST\t\r \u00C2\u00A0.....................................................\t\r \u00C2\u00A069\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A026.\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0RATIO\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0POPULATION\t\r \u00C2\u00A0AT\t\r \u00C2\u00A050%\t\r \u00C2\u00A0RISK\t\r \u00C2\u00A0TO\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0PREVALENCE\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0IN\t\r \u00C2\u00A04\t\r \u00C2\u00A0DIFFERENT\t\r \u00C2\u00A0POPULATIONS\t\r \u00C2\u00A0...\t\r \u00C2\u00A073\t\r \u00C2\u00A0FIGURE\t\r \u00C2\u00A027.\t\r \u00C2\u00A0STEPS\t\r \u00C2\u00A0TO\t\r \u00C2\u00A0BE\t\r \u00C2\u00A0TAKEN\t\r \u00C2\u00A0TO\t\r \u00C2\u00A0CONVERT\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0RESEARCH\t\r \u00C2\u00A0DATABASE\t\r \u00C2\u00A0INTO\t\r \u00C2\u00A0A\t\r \u00C2\u00A0REGISTER\t\r \u00C2\u00A0FOR\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0............\t\r \u00C2\u00A082\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 ix\t\r \u00C2\u00A0 Acknowledgements\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 First,\t\r \u00C2\u00A0I\t\r \u00C2\u00A0would\t\r \u00C2\u00A0like\t\r \u00C2\u00A0to\t\r \u00C2\u00A0acknowledge\t\r \u00C2\u00A0my\t\r \u00C2\u00A0thesis\t\r \u00C2\u00A0supervisor,\t\r \u00C2\u00A0Dr.\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0Hayden,\t\r \u00C2\u00A0for\t\r \u00C2\u00A0providing\t\r \u00C2\u00A0me\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0opportunity\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reach\t\r \u00C2\u00A0my\t\r \u00C2\u00A0potential.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A0accepting\t\r \u00C2\u00A0excellence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0nothing\t\r \u00C2\u00A0less\t\r \u00C2\u00A0is\t\r \u00C2\u00A0Dr.\t\r \u00C2\u00A0Hayden\u00E2\u0080\u0099s\t\r \u00C2\u00A0way\t\r \u00C2\u00A0and\t\r \u00C2\u00A0I\t\r \u00C2\u00A0know\t\r \u00C2\u00A0that\t\r \u00C2\u00A0his\t\r \u00C2\u00A0influence\t\r \u00C2\u00A0will\t\r \u00C2\u00A0change\t\r \u00C2\u00A0the\t\r \u00C2\u00A0way\t\r \u00C2\u00A0that\t\r \u00C2\u00A0I\t\r \u00C2\u00A0analyze\t\r \u00C2\u00A0and\t\r \u00C2\u00A0attempt\t\r \u00C2\u00A0to\t\r \u00C2\u00A0solve\t\r \u00C2\u00A0problems\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0rest\t\r \u00C2\u00A0of\t\r \u00C2\u00A0my\t\r \u00C2\u00A0life.\t\r \u00C2\u00A0\t\r \u00C2\u00A0I\t\r \u00C2\u00A0would\t\r \u00C2\u00A0also\t\r \u00C2\u00A0like\t\r \u00C2\u00A0to\t\r \u00C2\u00A0thank\t\r \u00C2\u00A0my\t\r \u00C2\u00A0supervisory\t\r \u00C2\u00A0committee,\t\r \u00C2\u00A0Susan\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Jan\t\r \u00C2\u00A0Friedman\t\r \u00C2\u00A0for\t\r \u00C2\u00A0taking\t\r \u00C2\u00A0the\t\r \u00C2\u00A0time\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0their\t\r \u00C2\u00A0extraordinarily\t\r \u00C2\u00A0busy\t\r \u00C2\u00A0schedules\t\r \u00C2\u00A0to\t\r \u00C2\u00A0show\t\r \u00C2\u00A0me\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ropes\t\r \u00C2\u00A0and\t\r \u00C2\u00A0critique\t\r \u00C2\u00A0my\t\r \u00C2\u00A0research.\t\r \u00C2\u00A0\t\r \u00C2\u00A0I\t\r \u00C2\u00A0would\t\r \u00C2\u00A0like\t\r \u00C2\u00A0to\t\r \u00C2\u00A0mention\t\r \u00C2\u00A0Alice\t\r \u00C2\u00A0Hawkins,\t\r \u00C2\u00A0who\t\r \u00C2\u00A0has\t\r \u00C2\u00A0gone\t\r \u00C2\u00A0above\t\r \u00C2\u00A0and\t\r \u00C2\u00A0beyond\t\r \u00C2\u00A0in\t\r \u00C2\u00A0ensuring\t\r \u00C2\u00A0my\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0in\t\r \u00C2\u00A0graduate\t\r \u00C2\u00A0school\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0successful.\t\r \u00C2\u00A0\t\r \u00C2\u00A0She\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0a\t\r \u00C2\u00A0mentor\t\r \u00C2\u00A0to\t\r \u00C2\u00A0me.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Thank\t\r \u00C2\u00A0you\t\r \u00C2\u00A0as\t\r \u00C2\u00A0well\t\r \u00C2\u00A0to\t\r \u00C2\u00A0Jennifer\t\r \u00C2\u00A0Collins,\t\r \u00C2\u00A0who\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0major\t\r \u00C2\u00A0assistance\t\r \u00C2\u00A0in\t\r \u00C2\u00A0setting\t\r \u00C2\u00A0up\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0database\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0project\t\r \u00C2\u00A0and\t\r \u00C2\u00A0to\t\r \u00C2\u00A0Alicia\t\r \u00C2\u00A0Semaka,\t\r \u00C2\u00A0for\t\r \u00C2\u00A0allowing\t\r \u00C2\u00A0me\t\r \u00C2\u00A0to\t\r \u00C2\u00A0work\t\r \u00C2\u00A0with\t\r \u00C2\u00A0her\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0summer\t\r \u00C2\u00A0student\t\r \u00C2\u00A0and\t\r \u00C2\u00A0for\t\r \u00C2\u00A0supporting\t\r \u00C2\u00A0my\t\r \u00C2\u00A0decision\t\r \u00C2\u00A0to\t\r \u00C2\u00A0join\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0graduate\t\r \u00C2\u00A0student.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Thank\t\r \u00C2\u00A0you\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0whole\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0for\t\r \u00C2\u00A0providing\t\r \u00C2\u00A0their\t\r \u00C2\u00A0expertise\t\r \u00C2\u00A0and\t\r \u00C2\u00A0insight\t\r \u00C2\u00A0whenever\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0and\t\r \u00C2\u00A0for\t\r \u00C2\u00A0being\t\r \u00C2\u00A0unconditionally\t\r \u00C2\u00A0helpful.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Finally,\t\r \u00C2\u00A0thank\t\r \u00C2\u00A0you\t\r \u00C2\u00A0to\t\r \u00C2\u00A0my\t\r \u00C2\u00A0family,\t\r \u00C2\u00A0mom,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0dad,\t\r \u00C2\u00A0Ben,\t\r \u00C2\u00A0Gillian,\t\r \u00C2\u00A0Sara,\t\r \u00C2\u00A0Andrew\t\r \u00C2\u00A0and\t\r \u00C2\u00A0David.\t\r \u00C2\u00A0\t\r \u00C2\u00A0You\t\r \u00C2\u00A0all\t\r \u00C2\u00A0inspire\t\r \u00C2\u00A0me\t\r \u00C2\u00A0and\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0role\t\r \u00C2\u00A0models\t\r \u00C2\u00A0for\t\r \u00C2\u00A0me\t\r \u00C2\u00A0since\t\r \u00C2\u00A0day\t\r \u00C2\u00A0one.\t\r \u00C2\u00A0\t\r \u00C2\u00A0I\t\r \u00C2\u00A0do\t\r \u00C2\u00A0not\t\r \u00C2\u00A0know\t\r \u00C2\u00A0where\t\r \u00C2\u00A0I\t\r \u00C2\u00A0would\t\r \u00C2\u00A0be\t\r \u00C2\u00A0without\t\r \u00C2\u00A0you.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 Introduction\t\r \u00C2\u00A0 Background\t\r \u00C2\u00A0 Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0(HD)\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0neurodegenerative\t\r \u00C2\u00A0disorder\t\r \u00C2\u00A0characterized\t\r \u00C2\u00A0by\t\r \u00C2\u00A0autosomal\t\r \u00C2\u00A0dominant\t\r \u00C2\u00A0inheritance.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Symptoms\t\r \u00C2\u00A0include\t\r \u00C2\u00A0psychiatric\t\r \u00C2\u00A0disturbances,\t\r \u00C2\u00A0cognitive\t\r \u00C2\u00A0decline\t\r \u00C2\u00A0and\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0abnormalities\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0chorea,\t\r \u00C2\u00A0dystonia\t\r \u00C2\u00A0and\t\r \u00C2\u00A0rigidity\t\r \u00C2\u00A0(Tabrizi\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Onset\t\r \u00C2\u00A0can\t\r \u00C2\u00A0occur\t\r \u00C2\u00A0at\t\r \u00C2\u00A0any\t\r \u00C2\u00A0time\t\r \u00C2\u00A0of\t\r \u00C2\u00A0life\t\r \u00C2\u00A0but\t\r \u00C2\u00A0most\t\r \u00C2\u00A0commonly\t\r \u00C2\u00A0arises\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mid\t\r \u00C2\u00A040\u00E2\u0080\u0099s\t\r \u00C2\u00A0(Kirkwood,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001).\t\r \u00C2\u00A0\t\r \u00C2\u00A0George\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0described\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A01872\t\r \u00C2\u00A0and\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0the\t\r \u00C2\u00A0autosomal\t\r \u00C2\u00A0dominant\t\r \u00C2\u00A0inheritance\t\r \u00C2\u00A0pattern\t\r \u00C2\u00A0(Huntington\t\r \u00C2\u00A0G,\t\r \u00C2\u00A01972).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A01987,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0test\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0became\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0this\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0restriction\t\r \u00C2\u00A0fragment\t\r \u00C2\u00A0length\t\r \u00C2\u00A0polymorphisms\t\r \u00C2\u00A0(RFLPs)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0analyzed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0multiple\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0results\t\r \u00C2\u00A0for\t\r \u00C2\u00A0one\t\r \u00C2\u00A0individual.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Certain\t\r \u00C2\u00A0RFLPs\t\r \u00C2\u00A0segregated\t\r \u00C2\u00A0only\t\r \u00C2\u00A0with\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Inferences\t\r \u00C2\u00A0could\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0be\t\r \u00C2\u00A0made\t\r \u00C2\u00A0on\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0the\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0carried\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0gene\t\r \u00C2\u00A0discovery\t\r \u00C2\u00A0(Gusella\t\r \u00C2\u00A01987).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Linkage\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0was\t\r \u00C2\u00A0mainly\t\r \u00C2\u00A0used\t\r \u00C2\u00A0for\t\r \u00C2\u00A0pre-\u00C2\u00AD\u00E2\u0080\u0090symptomatic\t\r \u00C2\u00A0(predictive)\t\r \u00C2\u00A0testing,\t\r \u00C2\u00A0but\t\r \u00C2\u00A0in\t\r \u00C2\u00A0some\t\r \u00C2\u00A0cases,\t\r \u00C2\u00A0aided\t\r \u00C2\u00A0in\t\r \u00C2\u00A0confirmation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0(S.\t\r \u00C2\u00A0Creighton,\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0communication,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0was\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0place\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0world\t\r \u00C2\u00A0to\t\r \u00C2\u00A0offer\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0using\t\r \u00C2\u00A0the\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0technique\t\r \u00C2\u00A0(Fox\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01989).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A01993,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0was\t\r \u00C2\u00A0identified\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0became\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0This\t\r \u00C2\u00A0test\t\r \u00C2\u00A0was\t\r \u00C2\u00A0used\t\r \u00C2\u00A0for\t\r \u00C2\u00A0both\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0and\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0purposes.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0are\t\r \u00C2\u00A0more\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0than\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0and\t\r \u00C2\u00A0contrary\t\r \u00C2\u00A0to\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0requiring\t\r \u00C2\u00A0the\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0multiple\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0undergoing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0are\t\r \u00C2\u00A0able\t\r \u00C2\u00A0to\t\r \u00C2\u00A0do\t\r \u00C2\u00A0so\t\r \u00C2\u00A0independently.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0that\t\r \u00C2\u00A0an\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0with\t\r \u00C2\u00A036\t\r \u00C2\u00A0or\t\r \u00C2\u00A0more\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HTT\t\r \u00C2\u00A0gene\t\r \u00C2\u00A0(4p16.3)\t\r \u00C2\u00A0will\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0become\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0her\t\r \u00C2\u00A0lifetime\t\r \u00C2\u00A0(MacMillan\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01993).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0data\t\r \u00C2\u00A0have\t\r \u00C2\u00A0led\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0classification\t\r \u00C2\u00A0of\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0sizes\t\r \u00C2\u00A0into\t\r \u00C2\u00A0four\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0ranges:\t\r \u00C2\u00A0fewer\t\r \u00C2\u00A0than\t\r \u00C2\u00A027\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0result\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0normal\t\r \u00C2\u00A0phenotype;\t\r \u00C2\u00A0intermediate\t\r \u00C2\u00A0alleles\t\r \u00C2\u00A0(IAs)\t\r \u00C2\u00A0comprise\t\r \u00C2\u00A0a\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A027\u00E2\u0080\u00932\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0IAs\t\r \u00C2\u00A0are\t\r \u00C2\u00A0below\t\r \u00C2\u00A0the\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeat\t\r \u00C2\u00A0range,\t\r \u00C2\u00A0but\t\r \u00C2\u00A0are\t\r \u00C2\u00A0thought\t\r \u00C2\u00A0to\t\r \u00C2\u00A0carry\t\r \u00C2\u00A0a\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0of\t\r \u00C2\u00A0expansion\t\r \u00C2\u00A0into\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0range\t\r \u00C2\u00A0within\t\r \u00C2\u00A0one\t\r \u00C2\u00A0generation;\t\r \u00C2\u00A036\u00E2\u0080\u009339\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0are\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 considered\t\r \u00C2\u00A0abnormal\t\r \u00C2\u00A0but\t\r \u00C2\u00A0are\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0reduced\t\r \u00C2\u00A0penetrance\t\r \u00C2\u00A0(RP)\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0for\t\r \u00C2\u00A0RP\t\r \u00C2\u00A0allele-\u00C2\u00AD\u00E2\u0080\u0090bearing\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0either\t\r \u00C2\u00A0very\t\r \u00C2\u00A0late\t\r \u00C2\u00A0or\t\r \u00C2\u00A0may\t\r \u00C2\u00A0not\t\r \u00C2\u00A0occur\t\r \u00C2\u00A0at\t\r \u00C2\u00A0all\t\r \u00C2\u00A0(Quarrell\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02007);\t\r \u00C2\u00A0forty\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0or\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0lifespan\t\r \u00C2\u00A0permitting\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0invariably\t\r \u00C2\u00A0give\t\r \u00C2\u00A0rise\t\r \u00C2\u00A0to\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Evidence\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0is\t\r \u00C2\u00A0inversely\t\r \u00C2\u00A0correlated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0individual\u00E2\u0080\u0099s\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeat\t\r \u00C2\u00A0length;\t\r \u00C2\u00A0an\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeat\t\r \u00C2\u00A0length\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0range\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0develop\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0later\t\r \u00C2\u00A0in\t\r \u00C2\u00A0life\t\r \u00C2\u00A0and\t\r \u00C2\u00A0vice\t\r \u00C2\u00A0versa\t\r \u00C2\u00A0(Langbehn,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02004).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0during\t\r \u00C2\u00A0a\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0period\t\r \u00C2\u00A0of\t\r \u00C2\u00A0time\t\r \u00C2\u00A0(Rothman\t\r \u00C2\u00A02002).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0the\t\r \u00C2\u00A0central\t\r \u00C2\u00A0focus\t\r \u00C2\u00A0of\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0on\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01,\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A01a,b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0new\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0and\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0studied\t\r \u00C2\u00A0less\t\r \u00C2\u00A0than\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0living\t\r \u00C2\u00A0during\t\r \u00C2\u00A0a\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0period\t\r \u00C2\u00A0of\t\r \u00C2\u00A0time\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0become\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0future\t\r \u00C2\u00A0(Rothman\t\r \u00C2\u00A02002).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0given\t\r \u00C2\u00A0little\t\r \u00C2\u00A0attention\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0on\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0or\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01a),\t\r \u00C2\u00A0and\t\r \u00C2\u00A0all\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0were\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0before\t\r \u00C2\u00A0either\t\r \u00C2\u00A0type\t\r \u00C2\u00A0of\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0was\t\r \u00C2\u00A0available\t\r \u00C2\u00A0(Barbeau\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01964,\t\r \u00C2\u00A0Shokeir,\t\r \u00C2\u00A01975).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Global\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0range\t\r \u00C2\u00A0from\t\r \u00C2\u00A00.01/100,000\t\r \u00C2\u00A0(1/10\t\r \u00C2\u00A0million)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0(Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01980),\t\r \u00C2\u00A0to\t\r \u00C2\u00A046.2/100,000\t\r \u00C2\u00A0(1/2,000)\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Mauritius\t\r \u00C2\u00A0(Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01981).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0extraordinarily\t\r \u00C2\u00A0high\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0699.2/100,000\t\r \u00C2\u00A0(1/143)\t\r \u00C2\u00A0was\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Maracaibo\t\r \u00C2\u00A0region\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Venezuela.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0abnormally\t\r \u00C2\u00A0high\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0along\t\r \u00C2\u00A0with\t\r \u00C2\u00A0those\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Mauritius\t\r \u00C2\u00A0and\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Genoa\t\r \u00C2\u00A0region\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01)\t\r \u00C2\u00A0have\t\r \u00C2\u00A0all\t\r \u00C2\u00A0been\t\r \u00C2\u00A0attributed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0extreme\t\r \u00C2\u00A0founder\t\r \u00C2\u00A0effects\t\r \u00C2\u00A0or\t\r \u00C2\u00A0insufficient\t\r \u00C2\u00A0sample\t\r \u00C2\u00A0population\t\r \u00C2\u00A0sizes\t\r \u00C2\u00A0(Young\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01986,\t\r \u00C2\u00A0Roccatagliata\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01983).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0average\t\r \u00C2\u00A0global\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0including\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Maracaibo\t\r \u00C2\u00A0is\t\r \u00C2\u00A011.2/100,000\t\r \u00C2\u00A0(1/8,929),\t\r \u00C2\u00A0while\t\r \u00C2\u00A0that\t\r \u00C2\u00A0excluding\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region\t\r \u00C2\u00A0is\t\r \u00C2\u00A05.1/100,000\t\r \u00C2\u00A0(1/19,608)\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0global\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0while\t\r \u00C2\u00A0excluding\t\r \u00C2\u00A0all\t\r \u00C2\u00A0three\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0disproportionately\t\r \u00C2\u00A0large\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0is\t\r \u00C2\u00A04.5/100,000\t\r \u00C2\u00A0(1/22,000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 considering\t\r \u00C2\u00A0only\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0after\t\r \u00C2\u00A0the\t\r \u00C2\u00A0availability\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0global\t\r \u00C2\u00A0average\t\r \u00C2\u00A0is\t\r \u00C2\u00A0again\t\r \u00C2\u00A05.1/100,000\t\r \u00C2\u00A0(1/19,608)\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01).\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A01.\t\r \u00C2\u00A0Estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0that\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0 after\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0(post\t\r \u00C2\u00A01993)\t\r \u00C2\u00A0 Region\t\r \u00C2\u00A0 Study\t\r \u00C2\u00A0year\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0(#/100,000)\t\r \u00C2\u00A0(1/X)\t\r \u00C2\u00A0 Incidence\t\r \u00C2\u00A0(#/million/year)\t\r \u00C2\u00A0 Pop.\t\r \u00C2\u00A0At\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0(#/100,000)\t\r \u00C2\u00A0 References\t\r \u00C2\u00A0Switzerland\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Austria\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10.0**\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10.0**\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 30.0*\t\r \u00C2\u00A0 (Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 42.0*\t\r \u00C2\u00A0 (Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0 1999\t\r \u00C2\u00A0 8.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 33.9\t\r \u00C2\u00A0 (Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009)\t\r \u00C2\u00A0Malta\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 11.8\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Gassivaro\t\r \u00C2\u00A0Gallo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0 1994-\u00C2\u00AD\u00E2\u0080\u00902002\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 4.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Ramos-\u00C2\u00AD\u00E2\u0080\u0090Arroyo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02004)\t\r \u00C2\u00A0 Greece\t\r \u00C2\u00A0 1995-\u00C2\u00AD\u00E2\u0080\u00902008\t\r \u00C2\u00A0 2.5-\u00C2\u00AD\u00E2\u0080\u00905.4\t\r \u00C2\u00A0 3.3\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Panas\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0New\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (McCusker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000)\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0 1997\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Adachi\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Nakashima\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0 2001\t\r \u00C2\u00A0 10.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 44.9\t\r \u00C2\u00A0 (Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010)\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 6.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 32.5\t\r \u00C2\u00A0 (Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000)\t\r \u00C2\u00A0Croatia\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Hecimovic\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002)\t\r \u00C2\u00A0Slovenia\t\r \u00C2\u00A0 2006\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Peterlin\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02008)\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Paradisi\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02008)\t\r \u00C2\u00A0Taiwan\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 Chen\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Lai\t\r \u00C2\u00A02010\t\r \u00C2\u00A0Mexico\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 4.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Alonso\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02009)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 5.9-\u00C2\u00AD\u00E2\u0080\u00906.5\t\r \u00C2\u00A0 6.1\t\r \u00C2\u00A0 37.5*\t\r \u00C2\u00A0 (Sackley\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0England\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 12.4\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 (Rawlins\t\r \u00C2\u00A02010)\t\r \u00C2\u00A0 Global\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5.1\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 34.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0*\t\r \u00C2\u00A0Indirect\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0that\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0**\t\r \u00C2\u00A0Published\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0not\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0exact,\t\r \u00C2\u00A0but\t\r \u00C2\u00A0approximate\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0and\t\r \u00C2\u00A0general\t\r \u00C2\u00A0population\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0appears\t\r \u00C2\u00A0to\t\r \u00C2\u00A0vary\t\r \u00C2\u00A0widely\t\r \u00C2\u00A0across\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0and\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A01,\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A02,\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A03\t\r \u00C2\u00A0and\t\r \u00C2\u00A0appendix\t\r \u00C2\u00A01a).\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0long-\u00C2\u00AD\u00E2\u0080\u0090standing\t\r \u00C2\u00A0question\t\r \u00C2\u00A0is\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0these\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0fluctuations\t\r \u00C2\u00A0are\t\r \u00C2\u00A0due\t\r \u00C2\u00A0primarily\t\r \u00C2\u00A0to\t\r \u00C2\u00A0variation\t\r \u00C2\u00A0in\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0precision\t\r \u00C2\u00A0or\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0true\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 (Myrianthopoulos\t\r \u00C2\u00A01966).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Evidence\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0that\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Asian\t\r \u00C2\u00A0and\t\r \u00C2\u00A0African\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0is\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0than\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0European\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A03)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0is\t\r \u00C2\u00A0thought\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0arisen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Europe\t\r \u00C2\u00A0(Warby\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0mean\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0for\t\r \u00C2\u00A0all\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0European\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0is\t\r \u00C2\u00A05.8/100,000\t\r \u00C2\u00A0(1/17,241)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0for\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Asian\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A00.62/100,000\t\r \u00C2\u00A0(1/161,290)\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A03).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0mean\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0all\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0African\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0is\t\r \u00C2\u00A02.8/100,000\t\r \u00C2\u00A0(1/35,714);\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0European\t\r \u00C2\u00A0average\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A03).\t\r \u00C2\u00A0\t\r \u00C2\u00A0However,\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0from\t\r \u00C2\u00A0African\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0range\t\r \u00C2\u00A0widely,\t\r \u00C2\u00A0from\t\r \u00C2\u00A00.01-\u00C2\u00AD\u00E2\u0080\u00907.0/100,000\t\r \u00C2\u00A0and\t\r \u00C2\u00A0all\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region\t\r \u00C2\u00A0are\t\r \u00C2\u00A0outdated\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further\t\r \u00C2\u00A0work\t\r \u00C2\u00A0is\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0required\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0gain\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0these\t\r \u00C2\u00A0populations.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A0two\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0have\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0taken\t\r \u00C2\u00A0place\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0first,\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Quebec\t\r \u00C2\u00A0in\t\r \u00C2\u00A01964,\t\r \u00C2\u00A0found\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A03.4/100,000\t\r \u00C2\u00A0(1/29,412)\t\r \u00C2\u00A0(Barbeau\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01964)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0second,\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Saskatchewan\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Manitoba\t\r \u00C2\u00A0in\t\r \u00C2\u00A01975,\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A08.4/100,000\t\r \u00C2\u00A0(1/11,905)\t\r \u00C2\u00A0(Shokeir\t\r \u00C2\u00A01975).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4.2$ 5.8$ 2.8$ 0.62$ 6.8$ Figure\t\r \u00C2\u00A01.\t\r \u00C2\u00A0Global\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0averages\t\r \u00C2\u00A0(#/100,000)\t\r \u00C2\u00A0by\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Americas:\t\r \u00C2\u00A04.2,\t\r \u00C2\u00A0 Europe:\t\r \u00C2\u00A05.8,\t\r \u00C2\u00A0Africa:\t\r \u00C2\u00A02.8,\t\r \u00C2\u00A0Asia:\t\r \u00C2\u00A00.62\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0&\t\r \u00C2\u00A0New\t\r \u00C2\u00A0Zealand:\t\r \u00C2\u00A06.8\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A01\t\r \u00C2\u00A0provides\t\r \u00C2\u00A0a\t\r \u00C2\u00A0full\t\r \u00C2\u00A0list\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0and\t\r \u00C2\u00A0their\t\r \u00C2\u00A0corresponding\t\r \u00C2\u00A0references\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902012:\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0Prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test \t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 Prevale nce\t\r \u00C2\u00A0(/1 00,000 )\t\r \u00C2\u00A0 Prevale nce\t\r \u00C2\u00A0(/1 00,000 )\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A03.\t\r \u00C2\u00A0Comparison\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0averages\t\r \u00C2\u00A0between\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Asian,\t\r \u00C2\u00A0African\t\r \u00C2\u00A0and\t\r \u00C2\u00A0 European\t\r \u00C2\u00A0descent.\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A02.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0average\t\r \u00C2\u00A0of\t\r \u00C2\u00A0all\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0on\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0Bar\t\r \u00C2\u00A0colours\t\r \u00C2\u00A0 represent\t\r \u00C2\u00A0the\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0techniques\t\r \u00C2\u00A0available\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r 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Austria \t\r \u00C2\u00A0 Germa ny\t\r \u00C2\u00A0 Japan\t\r \u00C2\u00A0( San-\u00C2\u00AD\u00E2\u0080\u0090in) \t\r \u00C2\u00A0 Switze rland\t\r \u00C2\u00A0 Austra lia\t\r \u00C2\u00A0 Croatia \t\r \u00C2\u00A0 Sloven ia\t\r \u00C2\u00A0 Taiwan \t\r \u00C2\u00A0 Greece \t\r \u00C2\u00A0 Mexico \t\r \u00C2\u00A0 Venezu ela\t\r \u00C2\u00A0 UK\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 0.1\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 1.50\t\r \u00C2\u00A0 0.01\t\r \u00C2\u00A0 6.4\t\r \u00C2\u00A0 7.0\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 0.8\t\r \u00C2\u00A0 6.8\t\r \u00C2\u00A0 5.3\t\r \u00C2\u00A0 4.2\t\r \u00C2\u00A0 0\t\r \u00C2\u00A01\t\r \u00C2\u00A0 2\t\r \u00C2\u00A03\t\r \u00C2\u00A0 4\t\r \u00C2\u00A05\t\r \u00C2\u00A0 6\t\r \u00C2\u00A07\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 Asian\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0 0.62\t\r \u00C2\u00A0 African\t\r \u00C2\u00A0descent\t\r \u00C2\u00A0\t\r \u00C2\u00A02.8\t\r \u00C2\u00A0\t\r \u00C2\u00A0 European\t\r \u00C2\u00A0descent\t\r \u00C2\u00A05.8\t\r \u00C2\u00A0Ethnic\t\r \u00C2\u00A0origin:\t\r \u00C2\u00A0Mean\t\r \u00C2\u00A0prevalence:\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902012:\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0Prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test \t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902012:\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0Prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test 1930\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0globally\t\r \u00C2\u00A0from\t\r \u00C2\u00A01.0\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Taiwan\t\r \u00C2\u00A0(Chen\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Lai,\t\r \u00C2\u00A02010)\t\r \u00C2\u00A0to\t\r \u00C2\u00A06.9\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0in\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0(Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001).\t\r \u00C2\u00A0\t\r \u00C2\u00A0To\t\r \u00C2\u00A0our\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0only\t\r \u00C2\u00A0seven\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0have\t\r \u00C2\u00A0taken\t\r \u00C2\u00A0place\t\r \u00C2\u00A0worldwide,\t\r \u00C2\u00A0two\t\r \u00C2\u00A0of\t\r \u00C2\u00A0which\t\r 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\u00C2\u00A0studies\t\r \u00C2\u00A0that\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0after\t\r \u00C2\u00A01993,\t\r \u00C2\u00A0is\t\r \u00C2\u00A05.7\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0has\t\r \u00C2\u00A0received\t\r \u00C2\u00A0little\t\r \u00C2\u00A0attention\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0With\t\r \u00C2\u00A0the\t\r \u00C2\u00A0exception\t\r \u00C2\u00A0of\t\r \u00C2\u00A0three\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0only\t\r \u00C2\u00A0from\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0exceptional\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0include\t\r \u00C2\u00A0one\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Netherlands,\t\r \u00C2\u00A0one\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Victoria,\t\r \u00C2\u00A0Australia,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0one\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland,\t\r \u00C2\u00A0all\t\r \u00C2\u00A0of\t\r \u00C2\u00A0which\t\r \u00C2\u00A0utilized\t\r \u00C2\u00A0electronic\t\r \u00C2\u00A0registries\t\r \u00C2\u00A0to\t\r \u00C2\u00A0calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0directly\t\r \u00C2\u00A0(Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011)1,\t\r \u00C2\u00A02,\t\r \u00C2\u00A03.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0the\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0multiplying\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A0three\t\r \u00C2\u00A0or\t\r \u00C2\u00A0by\t\r \u00C2\u00A0five\t\r \u00C2\u00A0depending\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0study\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0(Taylor\t\r \u00C2\u00A01994,\t\r \u00C2\u00A0Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999,\t\r \u00C2\u00A0Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Goizet\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Multiplying\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A0five\t\r \u00C2\u00A0was\t\r \u00C2\u00A0provoked\t\r \u00C2\u00A0by\t\r \u00C2\u00A0Conneally\u00E2\u0080\u0099s\t\r \u00C2\u00A0theory\t\r \u00C2\u00A0(Conneally,\t\r \u00C2\u00A01984).\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0theory\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0that\t\r \u00C2\u00A0each\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0has\t\r \u00C2\u00A0on\t\r \u00C2\u00A0average\t\r \u00C2\u00A0five\t\r \u00C2\u00A0first-\u00C2\u00AD\u00E2\u0080\u0090degree\t\r \u00C2\u00A0relatives;\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0for\t\r \u00C2\u00A0every\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0there\t\r \u00C2\u00A0are\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0five\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A0one\t\r \u00C2\u00A0study\t\r \u00C2\u00A0has\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0by\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0population\t\r \u00C2\u00A0by\t\r \u00C2\u00A0three\t\r \u00C2\u00A0and\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0an\t\r \u00C2\u00A0explanation\t\r \u00C2\u00A0as\t\r \u00C2\u00A0to\t\r \u00C2\u00A0why\t\r \u00C2\u00A0this\t\r \u00C2\u00A0number\t\r \u00C2\u00A0was\t\r \u00C2\u00A0chosen\t\r \u00C2\u00A0(Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999).\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0full\t\r \u00C2\u00A0review\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0has\t\r \u00C2\u00A0yet\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0studied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0There\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0several\t\r \u00C2\u00A0accounts\t\r \u00C2\u00A0worldwide\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Caucasian\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0(Taylor\t\r \u00C2\u00A01994,\t\r \u00C2\u00A0Lacconne\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999,\t\r \u00C2\u00A0Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Goizet\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A01\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Leiden\t\r \u00C2\u00A0roster\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0containing\t\r \u00C2\u00A0information\t\r \u00C2\u00A0on\t\r \u00C2\u00A0every\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0who\t\r \u00C2\u00A0attended\t\r \u00C2\u00A0one\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0eight\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0departments\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0and\t\r \u00C2\u00A0counseling\t\r \u00C2\u00A0(Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000).\t\r \u00C2\u00A02\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0and\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0This\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0began\t\r \u00C2\u00A0in\t\r \u00C2\u00A01950\t\r \u00C2\u00A0and\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0up\t\r \u00C2\u00A0kept\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0since\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A03\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0register\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0and\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0This\t\r \u00C2\u00A0register\t\r \u00C2\u00A0began\t\r \u00C2\u00A0in\t\r \u00C2\u00A01976\t\r \u00C2\u00A0and\t\r \u00C2\u00A0only\t\r \u00C2\u00A0contains\t\r \u00C2\u00A0data\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A02005\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0software\t\r \u00C2\u00A0incompatibility\t\r \u00C2\u00A0issues\t\r \u00C2\u00A0involving\t\r \u00C2\u00A0the\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0database\t\r \u00C2\u00A0system\t\r \u00C2\u00A0(Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 2003,\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010,\t\r \u00C2\u00A0Bernhardt\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Uptake\t\r \u00C2\u00A0is\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0expressed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0percentage\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Reports\t\r \u00C2\u00A0of\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0range\t\r \u00C2\u00A0from\t\r \u00C2\u00A03%\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Germany,\t\r \u00C2\u00A0Switzerland\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Austria\t\r \u00C2\u00A0(Lacconne\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999),\t\r \u00C2\u00A0to\t\r \u00C2\u00A025%\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0(Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A021%\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0years\t\r \u00C2\u00A01987-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0stated\t\r \u00C2\u00A0above,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk,\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0exception\t\r \u00C2\u00A0of\t\r \u00C2\u00A0few\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0assumed\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0lending\t\r \u00C2\u00A0a\t\r \u00C2\u00A0high\t\r \u00C2\u00A0possibility\t\r \u00C2\u00A0for\t\r \u00C2\u00A0inaccuracy\t\r \u00C2\u00A0in\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0proposed\t\r \u00C2\u00A0that\t\r \u00C2\u00A0all\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0accounts\t\r \u00C2\u00A0of\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0were\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0overestimated.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0report\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0that\t\r \u00C2\u00A0every\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0failed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0take\t\r \u00C2\u00A0into\t\r \u00C2\u00A0account\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0During\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0may\t\r \u00C2\u00A0change,\t\r \u00C2\u00A0thereby\t\r \u00C2\u00A0altering\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0results.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0a\t\r \u00C2\u00A0formula\t\r \u00C2\u00A0that\t\r \u00C2\u00A0would\t\r \u00C2\u00A0account\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period\t\r \u00C2\u00A0and\t\r \u00C2\u00A0showed\t\r \u00C2\u00A0that\t\r \u00C2\u00A0by\t\r \u00C2\u00A0using\t\r \u00C2\u00A0this\t\r \u00C2\u00A0formula,\t\r \u00C2\u00A0when\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0conventional\t\r \u00C2\u00A0methods,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0had\t\r \u00C2\u00A0decreased\t\r \u00C2\u00A0by\t\r \u00C2\u00A0nearly\t\r \u00C2\u00A0half.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 Rationale\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1.\t\r \u00C2\u00A0Updated\t\r \u00C2\u00A0and\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0are\t\r \u00C2\u00A0required:\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0to\t\r \u00C2\u00A0acquire\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0Firstly,\t\r \u00C2\u00A0these\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0allow\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0appropriate\t\r \u00C2\u00A0planning\t\r \u00C2\u00A0of\t\r \u00C2\u00A0services\t\r \u00C2\u00A0and\t\r \u00C2\u00A0allocation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0resources\t\r \u00C2\u00A0for\t\r \u00C2\u00A0those\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0in\t\r \u00C2\u00A0need.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Furthermore,\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0allow\t\r \u00C2\u00A0for\t\r \u00C2\u00A0meaningful\t\r \u00C2\u00A0comparisons\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0across\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0and\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0With\t\r \u00C2\u00A0a\t\r \u00C2\u00A0clearer\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0true\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0amid\t\r \u00C2\u00A0discrete\t\r \u00C2\u00A0populations,\t\r \u00C2\u00A0efforts\t\r \u00C2\u00A0towards\t\r \u00C2\u00A0studying\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0that\t\r \u00C2\u00A0underlie\t\r \u00C2\u00A0these\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0more\t\r \u00C2\u00A0efficiently\t\r \u00C2\u00A0designed\t\r \u00C2\u00A0(Warby\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Prevalence:\t\r \u00C2\u00A0The\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0were\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01a).\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0are\t\r \u00C2\u00A0outdated\t\r \u00C2\u00A0and\t\r \u00C2\u00A0cannot\t\r \u00C2\u00A0be\t\r \u00C2\u00A0meaningfully\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0modern\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0has\t\r \u00C2\u00A0since\t\r \u00C2\u00A0improved.\t\r \u00C2\u00A0\t\r \u00C2\u00A0However,\t\r \u00C2\u00A0when\t\r \u00C2\u00A0comparing\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0post-\u00C2\u00AD\u00E2\u0080\u00901993,\t\r \u00C2\u00A0after\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0test\t\r \u00C2\u00A0became\t\r \u00C2\u00A0available,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0that\t\r \u00C2\u00A0even\t\r \u00C2\u00A0these\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0vary\t\r \u00C2\u00A0widely\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01,\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A02).\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0uncertain\t\r \u00C2\u00A0as\t\r \u00C2\u00A0to\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0the\t\r \u00C2\u00A0bulk\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0variation\t\r \u00C2\u00A0is\t\r \u00C2\u00A0due\t\r \u00C2\u00A0primarily\t\r \u00C2\u00A0to\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0precision\t\r \u00C2\u00A0or\t\r \u00C2\u00A0to\t\r \u00C2\u00A0true\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0across\t\r \u00C2\u00A0populations.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0the\t\r \u00C2\u00A0possibility\t\r \u00C2\u00A0of\t\r \u00C2\u00A0under-\u00C2\u00AD\u00E2\u0080\u0090ascertainment,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0many\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0as\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0should\t\r \u00C2\u00A0be\t\r \u00C2\u00A0used.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0search\t\r \u00C2\u00A0for\t\r \u00C2\u00A0secondary\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0(i.e.\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0found\t\r \u00C2\u00A0via\t\r \u00C2\u00A0family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0as\t\r \u00C2\u00A0opposed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review)\t\r \u00C2\u00A0is\t\r \u00C2\u00A0also\t\r \u00C2\u00A0essential\t\r \u00C2\u00A0(Harper,\t\r \u00C2\u00A01992,\t\r \u00C2\u00A0Levy\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Fenigold,\t\r \u00C2\u00A02000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0post-\u00C2\u00AD\u00E2\u0080\u00901993\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0used\t\r \u00C2\u00A0less\t\r \u00C2\u00A0than\t\r \u00C2\u00A0three\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources,\t\r \u00C2\u00A0with\t\r \u00C2\u00A0three\t\r \u00C2\u00A0being\t\r \u00C2\u00A0the\t\r \u00C2\u00A0maximum\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0used\t\r \u00C2\u00A0(Peterlin\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Furthermore,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0study\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0in\t\r 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\u00C2\u00A0A\t\r \u00C2\u00A0thorough\t\r \u00C2\u00A0multi-\u00C2\u00AD\u00E2\u0080\u0090source\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0study\t\r \u00C2\u00A0is\t\r \u00C2\u00A0required\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0an\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0and\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0capable\t\r \u00C2\u00A0of\t\r \u00C2\u00A0setting\t\r \u00C2\u00A0a\t\r \u00C2\u00A0precedent\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 9\t\r \u00C2\u00A0 for\t\r \u00C2\u00A0future\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0and\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0with\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0to\t\r \u00C2\u00A0findings\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0regions.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Incidence:\t\r \u00C2\u00A0The\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0was\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0in\t\r \u00C2\u00A02008\t\r \u00C2\u00A0(Sackley,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A0seven\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date,\t\r \u00C2\u00A0two\t\r \u00C2\u00A0of\t\r \u00C2\u00A0which\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0before\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0became\t\r \u00C2\u00A0available\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0One\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0is\t\r \u00C2\u00A0from\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0and\t\r \u00C2\u00A0included\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0from\t\r \u00C2\u00A01993-\u00C2\u00AD\u00E2\u0080\u00901999;\t\r \u00C2\u00A0all\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0were\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0(Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0maintain\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0in\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0and\t\r \u00C2\u00A0to\t\r \u00C2\u00A0observe\t\r \u00C2\u00A0variation\t\r \u00C2\u00A0in\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0that\t\r \u00C2\u00A0these\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0are\t\r \u00C2\u00A0updated.\t\r \u00C2\u00A0\t\r \u00C2\u00A0With\t\r \u00C2\u00A012\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0available\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0data\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0at\t\r \u00C2\u00A0this\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0 Population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk:\t\r \u00C2\u00A0Worldwide\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0are\t\r \u00C2\u00A0largely\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0theories\t\r \u00C2\u00A0and\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0that\t\r \u00C2\u00A0do\t\r \u00C2\u00A0not\t\r \u00C2\u00A0pertain\t\r \u00C2\u00A0to\t\r \u00C2\u00A0any\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A01).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0familial\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0are\t\r \u00C2\u00A0often\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0their\t\r \u00C2\u00A0family\t\r \u00C2\u00A0structure\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0component\t\r \u00C2\u00A0of\t\r \u00C2\u00A0their\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0records.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0pedigrees,\t\r \u00C2\u00A0which\t\r \u00C2\u00A0are\t\r \u00C2\u00A0necessary\t\r \u00C2\u00A0for\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk,\t\r \u00C2\u00A0are\t\r \u00C2\u00A0readily\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Knowledge\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0and\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0is\t\r \u00C2\u00A0essential\t\r \u00C2\u00A0in\t\r \u00C2\u00A0care\t\r \u00C2\u00A0and\t\r \u00C2\u00A0service\t\r \u00C2\u00A0planning\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0should\t\r \u00C2\u00A0be\t\r \u00C2\u00A0studied\t\r \u00C2\u00A0empirically\t\r \u00C2\u00A0from\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0a\t\r \u00C2\u00A0comparison\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predicted\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0average\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0first-\u00C2\u00AD\u00E2\u0080\u0090degree\t\r \u00C2\u00A0relatives\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Furthermore,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0first-\u00C2\u00AD\u00E2\u0080\u0090degree\t\r \u00C2\u00A0relatives\t\r \u00C2\u00A0varies\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0between\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0(CIA\t\r \u00C2\u00A0World\t\r \u00C2\u00A0Factbook\t\r \u00C2\u00A02012),\t\r \u00C2\u00A0and\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0evidence\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0that\t\r \u00C2\u00A0multiplying\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0factor\t\r \u00C2\u00A0of\t\r \u00C2\u00A04.2\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0as\t\r \u00C2\u00A0opposed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0notion\t\r \u00C2\u00A0of\t\r \u00C2\u00A05\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0more\t\r \u00C2\u00A0accurate.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0to\t\r \u00C2\u00A0update\t\r \u00C2\u00A0the\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0uptake.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Firstly,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0equation\t\r \u00C2\u00A0for\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0has\t\r \u00C2\u00A0changed\t\r \u00C2\u00A0since\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0latest\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003),\t\r \u00C2\u00A0and\t\r \u00C2\u00A0second,\t\r \u00C2\u00A0empirical\t\r \u00C2\u00A0evidence\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0will\t\r \u00C2\u00A0become\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r 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\u00C2\u00A0British\t\r \u00C2\u00A0Columbia.\t\r \u00C2\u00A0One\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0is\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0performing\t\r \u00C2\u00A0all\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0entire\t\r \u00C2\u00A0province\t\r \u00C2\u00A0and\t\r \u00C2\u00A0one\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0serves\t\r \u00C2\u00A0as\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0care\t\r \u00C2\u00A0hub\t\r \u00C2\u00A0for\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0every\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0diagnosis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0ascertained.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further,\t\r \u00C2\u00A0in\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0was\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0that\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0had\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0a\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0total\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0(predictive\t\r \u00C2\u00A0and\t\r \u00C2\u00A0diagnostic)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0proportional\t\r \u00C2\u00A0to\t\r \u00C2\u00A0its\t\r \u00C2\u00A0population\t\r \u00C2\u00A0size,\t\r \u00C2\u00A0than\t\r \u00C2\u00A0any\t\r \u00C2\u00A0other\t\r \u00C2\u00A0province\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A0however,\t\r \u00C2\u00A0Quebec\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Alberta\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Having\t\r \u00C2\u00A0been\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0province\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0population\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0awareness\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0test\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0other\t\r \u00C2\u00A0provinces.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0further\t\r \u00C2\u00A0emphasizes\t\r \u00C2\u00A0the\t\r \u00C2\u00A0high\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0for\t\r \u00C2\u00A0full\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0Further,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0global\t\r \u00C2\u00A0hub\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0and\t\r \u00C2\u00A0care\t\r \u00C2\u00A0and\t\r \u00C2\u00A0as\t\r \u00C2\u00A0there\t\r \u00C2\u00A0are\t\r \u00C2\u00A0many\t\r \u00C2\u00A0opportunities\t\r \u00C2\u00A0for\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0families\t\r \u00C2\u00A0to\t\r \u00C2\u00A0participate\t\r \u00C2\u00A0in\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0trials\t\r \u00C2\u00A0and\t\r \u00C2\u00A0other\t\r \u00C2\u00A0types\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research,\t\r \u00C2\u00A0members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0more\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0seek\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0attention\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province\t\r \u00C2\u00A0than\t\r \u00C2\u00A0in\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0offering\t\r \u00C2\u00A0no\t\r \u00C2\u00A0such\t\r \u00C2\u00A0services\t\r \u00C2\u00A0or\t\r \u00C2\u00A0opportunities;\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0has\t\r \u00C2\u00A0a\t\r \u00C2\u00A0specialized\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0equipped\t\r \u00C2\u00A0with\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0counsellors,\t\r \u00C2\u00A0social\t\r \u00C2\u00A0workers,\t\r \u00C2\u00A0neurologists,\t\r \u00C2\u00A0psychiatrists,\t\r \u00C2\u00A0geneticists\t\r \u00C2\u00A0and\t\r \u00C2\u00A0an\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0research\t\r \u00C2\u00A0team,\t\r \u00C2\u00A0all\t\r \u00C2\u00A0with\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0challenges.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Furthermore,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0improved\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0consisting\t\r \u00C2\u00A0of\t\r \u00C2\u00A0500,000\t\r \u00C2\u00A0to\t\r \u00C2\u00A05,000,000\t\r \u00C2\u00A0people.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Populations\t\r \u00C2\u00A0smaller\t\r \u00C2\u00A0than\t\r \u00C2\u00A0this\t\r \u00C2\u00A0are\t\r \u00C2\u00A0thought\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0vulnerable\t\r \u00C2\u00A0to\t\r \u00C2\u00A0skewing\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0large\t\r \u00C2\u00A0families\t\r \u00C2\u00A0and\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0than\t\r \u00C2\u00A0this,\t\r \u00C2\u00A0vulnerable\t\r \u00C2\u00A0to\t\r \u00C2\u00A0incomplete\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0(Harper,\t\r \u00C2\u00A01992,\t\r \u00C2\u00A0Levy\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Feingold,\t\r \u00C2\u00A02000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0population\t\r \u00C2\u00A0is\t\r \u00C2\u00A0within\t\r \u00C2\u00A0these\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0limits\t\r \u00C2\u00A0(British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Statistics,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 11\t\r \u00C2\u00A0 Specific\t\r \u00C2\u00A0objectives\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0appropriately\t\r \u00C2\u00A0characterize\t\r \u00C2\u00A0the\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0posed\t\r \u00C2\u00A0three\t\r \u00C2\u00A0main\t\r \u00C2\u00A0objectives:\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1. Calculate\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0The\t\r \u00C2\u00A0first\t\r \u00C2\u00A0objective\t\r \u00C2\u00A0was\t\r \u00C2\u00A0to\t\r \u00C2\u00A0assess\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0and\t\r \u00C2\u00A0approximate\t\r \u00C2\u00A0geographical\t\r \u00C2\u00A0location\t\r \u00C2\u00A0of\t\r \u00C2\u00A0symptomatic\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0on\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 2. Calculate\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0The\t\r \u00C2\u00A0second\t\r \u00C2\u00A0objective\t\r \u00C2\u00A0was\t\r \u00C2\u00A0to\t\r \u00C2\u00A0assess\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0new\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r \u00C2\u00A0between\t\r \u00C2\u00A0January\t\r \u00C2\u00A01,\t\r \u00C2\u00A02001,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0December\t\r \u00C2\u00A031,\t\r \u00C2\u00A02011,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test-\u00C2\u00AD\u00E2\u0080\u0090rate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000\t\r \u00C2\u00A0and\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 3. Calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0The\t\r \u00C2\u00A0third\t\r \u00C2\u00A0objective\t\r \u00C2\u00A0was\t\r \u00C2\u00A0to\t\r \u00C2\u00A0assess\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0people\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0on\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0develop\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0future.\t\r \u00C2\u00A0\t\r \u00C2\u00A0There\t\r \u00C2\u00A0are\t\r \u00C2\u00A0two\t\r \u00C2\u00A0levels\t\r \u00C2\u00A0of\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0category,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0(i.e.\t\r \u00C2\u00A025%\t\r \u00C2\u00A0or\t\r \u00C2\u00A050%)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0categories\t\r \u00C2\u00A0after\t\r \u00C2\u00A0accounting\t\r \u00C2\u00A0for\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0objective\t\r \u00C2\u00A0also\t\r \u00C2\u00A0included\t\r \u00C2\u00A0an\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0and\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0between\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 12\t\r \u00C2\u00A0 Methodology\t\r \u00C2\u00A0 Epidemiology:\t\r \u00C2\u00A0definitions\t\r \u00C2\u00A0Epidemiology\t\r \u00C2\u00A0is\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0branch\t\r \u00C2\u00A0of\t\r \u00C2\u00A0medicine\t\r \u00C2\u00A0that\t\r \u00C2\u00A0deals\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence,\t\r \u00C2\u00A0distribution,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0control\t\r \u00C2\u00A0of\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0and\t\r \u00C2\u00A0other\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0relating\t\r \u00C2\u00A0to\t\r \u00C2\u00A0health\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0(J.\t\r \u00C2\u00A0Simpson\t\r \u00C2\u00A01989).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0are\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0commonly\t\r \u00C2\u00A0studied\t\r \u00C2\u00A0for\t\r \u00C2\u00A0disease.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0people\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0a\t\r \u00C2\u00A0point\t\r \u00C2\u00A0in\t\r \u00C2\u00A0time\t\r \u00C2\u00A0or\t\r \u00C2\u00A0over\t\r \u00C2\u00A0a\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A0time\t\r \u00C2\u00A0(Rothman\t\r \u00C2\u00A02002);\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0often\t\r \u00C2\u00A0expressed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0per\t\r \u00C2\u00A0100,000\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0or\t\r \u00C2\u00A01\t\r \u00C2\u00A0over\t\r \u00C2\u00A0X\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0people\t\r \u00C2\u00A0where\t\r \u00C2\u00A0X\t\r \u00C2\u00A0indicates\t\r \u00C2\u00A0the\t\r \u00C2\u00A0average\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0that\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0detect\t\r \u00C2\u00A0one\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0case.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0people\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0newly\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0new\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0(Rothman\t\r \u00C2\u00A02002);\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0often\t\r \u00C2\u00A0expressed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0newly\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0(incident\t\r \u00C2\u00A0cases)\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0per\t\r \u00C2\u00A0year.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0refers\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0a\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0point\t\r \u00C2\u00A0in,\t\r \u00C2\u00A0or\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of,\t\r \u00C2\u00A0time\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0become\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0(Rothman\t\r \u00C2\u00A02002).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Similar\t\r \u00C2\u00A0to\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0is\t\r \u00C2\u00A0often\t\r \u00C2\u00A0expressed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0at-\u00C2\u00AD\u00E2\u0080\u0090risk\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0per\t\r \u00C2\u00A0100,000\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0or\t\r \u00C2\u00A01\t\r \u00C2\u00A0over\t\r \u00C2\u00A0X\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0people.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0of\t\r \u00C2\u00A0acquiring\t\r \u00C2\u00A0a\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0depends\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetics,\t\r \u00C2\u00A0transmission\t\r \u00C2\u00A0patterns\t\r \u00C2\u00A0and\t\r \u00C2\u00A0other\t\r \u00C2\u00A0external\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0related\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0at\t\r \u00C2\u00A0hand.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0largely\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Taylor\t\r \u00C2\u00A01994,\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Goizet\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0doing\t\r \u00C2\u00A0so,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ratios\t\r \u00C2\u00A0of\t\r \u00C2\u00A01:3,\t\r \u00C2\u00A01:5\t\r \u00C2\u00A0and\t\r \u00C2\u00A0more\t\r \u00C2\u00A0recently\t\r \u00C2\u00A01:4.2\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009)\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected:\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0have\t\r \u00C2\u00A0largely\t\r \u00C2\u00A0been\t\r \u00C2\u00A0used.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0explanation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A01:5\t\r \u00C2\u00A0ratio\t\r \u00C2\u00A0is\t\r \u00C2\u00A0as\t\r \u00C2\u00A0follows.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0theory,\t\r \u00C2\u00A0\u00C2\u00BD\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0will\t\r \u00C2\u00A0carry\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0expansion.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0third\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0\u00C2\u00BD\t\r \u00C2\u00A0will\t\r \u00C2\u00A0be\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0at\t\r \u00C2\u00A0any\t\r \u00C2\u00A0given\t\r \u00C2\u00A0time,\t\r \u00C2\u00A0leaving\t\r \u00C2\u00A0two\t\r \u00C2\u00A0thirds\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0However,\t\r \u00C2\u00A0none\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0other\t\r \u00C2\u00A0\u00C2\u00BD\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0(who\t\r 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\u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A04.2\t\r \u00C2\u00A0ratio\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0two\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0via\t\r \u00C2\u00A0empirical\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 13\t\r \u00C2\u00A0 data\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0may\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0a\t\r \u00C2\u00A0more\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0measurement.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Population\t\r \u00C2\u00A0under\t\r \u00C2\u00A0review:\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0The\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0population\t\r \u00C2\u00A0in\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0of\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia,\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0(BC).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0for\t\r \u00C2\u00A02012\t\r \u00C2\u00A0as\t\r \u00C2\u00A0projected\t\r \u00C2\u00A0by\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0is\t\r \u00C2\u00A04,609,659\t\r \u00C2\u00A0(Statistics\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0last\t\r \u00C2\u00A0census\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0in\t\r \u00C2\u00A02011,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0actual\t\r \u00C2\u00A0(non-\u00C2\u00AD\u00E2\u0080\u0090projected)\t\r \u00C2\u00A0census\t\r \u00C2\u00A0population\t\r \u00C2\u00A0is\t\r \u00C2\u00A0not\t\r \u00C2\u00A0yet\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0assess\t\r \u00C2\u00A0the\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0was\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0in\t\r \u00C2\u00A0two\t\r \u00C2\u00A0ways.\t\r \u00C2\u00A0\t\r \u00C2\u00A0First,\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0into\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u0098Urban\u00E2\u0080\u0099\t\r \u00C2\u00A0regions.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0away\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0were\t\r \u00C2\u00A0designated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0category.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Cities\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0or\t\r \u00C2\u00A0less\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car,\t\r \u00C2\u00A0were\t\r \u00C2\u00A0designated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0category.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0definition\t\r \u00C2\u00A0renders\t\r \u00C2\u00A0some\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0as\t\r \u00C2\u00A0being\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0areas.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Although\t\r \u00C2\u00A0this\t\r \u00C2\u00A0may\t\r \u00C2\u00A0seem\t\r \u00C2\u00A0counterintuitive,\t\r \u00C2\u00A0these\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0categorizations\t\r \u00C2\u00A0were\t\r \u00C2\u00A0chosen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0mirror\t\r \u00C2\u00A0the\t\r \u00C2\u00A0convenience\t\r \u00C2\u00A0in\t\r \u00C2\u00A0accessing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Individuals\t\r \u00C2\u00A0requiring\t\r \u00C2\u00A0specialized\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0care\t\r \u00C2\u00A0or\t\r \u00C2\u00A0wishing\t\r \u00C2\u00A0to\t\r \u00C2\u00A0take\t\r \u00C2\u00A0part\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0are\t\r \u00C2\u00A0required\t\r \u00C2\u00A0to\t\r \u00C2\u00A0commute\t\r \u00C2\u00A0to\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0to\t\r \u00C2\u00A0do\t\r \u00C2\u00A0so.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0these\t\r \u00C2\u00A0opportunities\t\r \u00C2\u00A0renders\t\r \u00C2\u00A0these\t\r \u00C2\u00A0seemingly\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0as\t\r \u00C2\u00A0being\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purpose\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0second\t\r \u00C2\u00A0categorization\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0dividing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0into\t\r \u00C2\u00A0its\t\r \u00C2\u00A0constitutive\t\r \u00C2\u00A0provincial\t\r \u00C2\u00A0health\t\r \u00C2\u00A0regions.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Provincial\t\r \u00C2\u00A0health\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0are\t\r \u00C2\u00A0legislated\t\r \u00C2\u00A0administrative\t\r \u00C2\u00A0areas\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0provincial\t\r \u00C2\u00A0ministries\t\r \u00C2\u00A0of\t\r \u00C2\u00A0health.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0represent\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0areas\t\r \u00C2\u00A0of\t\r \u00C2\u00A0responsibility\t\r \u00C2\u00A0for\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0boards\t\r \u00C2\u00A0or\t\r \u00C2\u00A0regional\t\r 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\u00C2\u00A0an\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patient:\t\r \u00C2\u00A0Age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0Calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0involves\t\r \u00C2\u00A0counting\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0at\t\r \u00C2\u00A0a\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0point\t\r \u00C2\u00A0in\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Therefore,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 14\t\r \u00C2\u00A0 imperative\t\r \u00C2\u00A0to\t\r \u00C2\u00A0define\t\r \u00C2\u00A0what\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0render\t\r \u00C2\u00A0a\t\r \u00C2\u00A0case\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Before\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0testing,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0diagnosis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0could\t\r \u00C2\u00A0only\t\r \u00C2\u00A0be\t\r \u00C2\u00A0made\t\r \u00C2\u00A0when\t\r \u00C2\u00A0characteristic\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0signs\t\r \u00C2\u00A0and\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0were\t\r \u00C2\u00A0present.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Psychiatric\t\r \u00C2\u00A0and\t\r \u00C2\u00A0cognitive\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0were\t\r \u00C2\u00A0often\t\r \u00C2\u00A0too\t\r \u00C2\u00A0general\t\r \u00C2\u00A0to\t\r \u00C2\u00A0entail\t\r \u00C2\u00A0a\t\r \u00C2\u00A0definitive\t\r \u00C2\u00A0diagnosis\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0own\t\r \u00C2\u00A0(F.\t\r \u00C2\u00A0O.\t\r \u00C2\u00A0Walker\t\r \u00C2\u00A02007).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0availability\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0in\t\r \u00C2\u00A0two\t\r \u00C2\u00A0ways\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Affected\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0either\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0\u00E2\u0080\u009Csymptomatic\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0positive\u00E2\u0080\u009D,\t\r \u00C2\u00A0or\t\r \u00C2\u00A0\u00E2\u0080\u009Cclinically\t\r \u00C2\u00A0diagnosed\u00E2\u0080\u009D.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Symptomatic\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0positive:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0\u00E2\u0080\u009Csymptomatic\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0positive\u00E2\u0080\u009D\t\r \u00C2\u00A0if\t\r \u00C2\u00A0they\t\r \u00C2\u00A0have\t\r \u00C2\u00A0received\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0result\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0\t\r \u00C2\u00A0(HTT\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0size\t\r \u00C2\u00A0\u00E2\u0089\u00A536)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0evidence,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0notes,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0member\t\r \u00C2\u00A0or\t\r \u00C2\u00A0caregiver,\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0begun\t\r \u00C2\u00A0to\t\r \u00C2\u00A0exhibit\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0no\t\r \u00C2\u00A0information\t\r \u00C2\u00A0is\t\r \u00C2\u00A0available\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0symptoms,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Unified\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Rating\t\r \u00C2\u00A0Scale\t\r \u00C2\u00A0(UHDRS)4\t\r \u00C2\u00A0assessment,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0date\t\r \u00C2\u00A0on\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0first\t\r \u00C2\u00A0received\t\r \u00C2\u00A0a\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A0\u00E2\u0089\u00A52\t\r \u00C2\u00A0was\t\r \u00C2\u00A0used\t\r \u00C2\u00A0to\t\r \u00C2\u00A0define\t\r \u00C2\u00A0the\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0score\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0value\t\r \u00C2\u00A0ranging\t\r \u00C2\u00A0from\t\r \u00C2\u00A00-\u00C2\u00AD\u00E2\u0080\u00904;\t\r \u00C2\u00A0zero\t\r \u00C2\u00A0means\t\r \u00C2\u00A0that\t\r \u00C2\u00A0no\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0are\t\r \u00C2\u00A0present\t\r 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\u00C2\u00A0progression\t\r \u00C2\u00A0of\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0(Orth\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Schwenke,\t\r \u00C2\u00A02011),\t\r \u00C2\u00A0a\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0motor\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0as\t\r \u00C2\u00A0this\t\r \u00C2\u00A0score\t\r \u00C2\u00A0has\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0sufficient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0detecting\t\r \u00C2\u00A0noticeable\t\r \u00C2\u00A0motor\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0in\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0practice\t\r \u00C2\u00A0(M.\t\r \u00C2\u00A0Hayden,\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0correspondence,\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r 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\u00C2\u00A0 Clinically\t\r \u00C2\u00A0diagnosed:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0clinically\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0if\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0clear\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0evidence\t\r \u00C2\u00A0(from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0notes,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0member\t\r \u00C2\u00A0or\t\r \u00C2\u00A0caregiver)\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0exhibiting\t\r 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\u00C2\u00A0neurologists,\t\r \u00C2\u00A0psychiatrists,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0social\t\r \u00C2\u00A0worker,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0counsellor\t\r \u00C2\u00A0work\t\r \u00C2\u00A0together\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0(Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0major\t\r \u00C2\u00A0hub\t\r \u00C2\u00A0for\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0research\t\r \u00C2\u00A0and\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0care;\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0families\t\r \u00C2\u00A0from\t\r \u00C2\u00A0across\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0and\t\r \u00C2\u00A0from\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r 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\u00C2\u00A0built\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Microsoft\t\r \u00C2\u00A0Access\t\r \u00C2\u00A0platform\t\r \u00C2\u00A0and\t\r \u00C2\u00A0pertinent\t\r \u00C2\u00A0information\t\r \u00C2\u00A0was\t\r \u00C2\u00A0extracted\t\r \u00C2\u00A0from\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0and\t\r \u00C2\u00A0entered\t\r \u00C2\u00A0into\t\r \u00C2\u00A0the\t\r \u00C2\u00A0database.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0extracted\t\r \u00C2\u00A0that\t\r \u00C2\u00A0was\t\r \u00C2\u00A0most\t\r \u00C2\u00A0relevant\t\r \u00C2\u00A0to\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 17\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk,\t\r \u00C2\u00A0included:\t\r \u00C2\u00A0province\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence,\t\r \u00C2\u00A0presence\t\r \u00C2\u00A0or\t\r \u00C2\u00A0absence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history,\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0living\t\r \u00C2\u00A0or\t\r \u00C2\u00A0deceased,\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death,\t\r \u00C2\u00A0age\t\r \u00C2\u00A0and\t\r \u00C2\u00A0year\t\r \u00C2\u00A0of\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset,\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0motor\t\r \u00C2\u00A0score\t\r \u00C2\u00A0assessment,\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0size,\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0status,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0and\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A02).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A02\t\r \u00C2\u00A0contains\t\r \u00C2\u00A0a\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0list\t\r \u00C2\u00A0and\t\r \u00C2\u00A0description\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0field\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0database.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A02.\t\r \u00C2\u00A0Fields\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Access\t\r \u00C2\u00A0database\t\r \u00C2\u00A0pertaining\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0 Field\t\r \u00C2\u00A0Name\t\r \u00C2\u00A0 Field\t\r \u00C2\u00A0Description\t\r \u00C2\u00A0Province\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Family\t\r \u00C2\u00A0History\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD?\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Yes\t\r \u00C2\u00A0\u00E2\u0080\u00A2 No\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Unknown\t\r \u00C2\u00A0Deceased?\t\r \u00C2\u00A0 Yes\t\r \u00C2\u00A0or\t\r \u00C2\u00A0No\t\r \u00C2\u00A0If\t\r \u00C2\u00A0yes,\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death\t\r \u00C2\u00A0 Motor\t\r \u00C2\u00A0age/year\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0age/year\t\r \u00C2\u00A0at\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0first\t\r \u00C2\u00A0noted\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0experienced\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Chorea/dystonia\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Rigidity\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Difficulty\t\r \u00C2\u00A0with\t\r \u00C2\u00A0balance\t\r \u00C2\u00A0Difficulty\t\r \u00C2\u00A0breathing/swallowing\t\r \u00C2\u00A0UHDRS?\t\r \u00C2\u00A0 Has\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0a\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0assessment?\t\r \u00C2\u00A0United\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Rating\t\r \u00C2\u00A0Scale:\t\r \u00C2\u00A0Developed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Study\t\r \u00C2\u00A0Group\t\r \u00C2\u00A0in\t\r \u00C2\u00A01996i\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0Assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0features\t\r \u00C2\u00A0to\t\r \u00C2\u00A0track\t\r 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\u00C2\u00A0 \u00E2\u0080\u00A2 25%\t\r \u00C2\u00A0AR\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Unknown\t\r \u00C2\u00A0AR\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Affected\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Pre\t\r \u00C2\u00A0Manifest\t\r \u00C2\u00A0(100%\t\r \u00C2\u00A0AR)\t\r \u00C2\u00A0Family\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0totals\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0family\t\r \u00C2\u00A0at\t\r \u00C2\u00A0each\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0category\t\r \u00C2\u00A0\u00E2\u0080\u00A2 BC\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0only\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0Not\t\r \u00C2\u00A0yet\t\r \u00C2\u00A0in\t\r \u00C2\u00A0database\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0living\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0family\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Not\t\r \u00C2\u00A0a\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0\u00E2\u0080\u00A2 BC\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0only\t\r \u00C2\u00A0\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0second\t\r \u00C2\u00A0method\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0was\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0results\t\r \u00C2\u00A0from\t\r \u00C2\u00A0every\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0that\t\r \u00C2\u00A0has\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0been\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0available\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A01993\t\r \u00C2\u00A0and\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0nearly\t\r \u00C2\u00A020\t\r \u00C2\u00A0years\t\r \u00C2\u00A0worth\t\r \u00C2\u00A0of\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0single\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0is\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0performing\t\r \u00C2\u00A0all\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0entire\t\r \u00C2\u00A0province.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0laboratory\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0Diagnostic\t\r \u00C2\u00A0Laboratory\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Children\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Women\u00E2\u0080\u0099s\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0in\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 18\t\r \u00C2\u00A0 Vancouver\t\r \u00C2\u00A0(C&W)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0reviewed.\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Genetic\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0between\t\r \u00C2\u00A01987-\u00C2\u00AD\u00E2\u0080\u00902001\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0and\t\r \u00C2\u00A0those\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0between\t\r \u00C2\u00A02002-\u00C2\u00AD\u00E2\u0080\u00902012\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0at\t\r \u00C2\u00A0C&W.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0hard\t\r \u00C2\u00A0copy\t\r \u00C2\u00A0test\t\r \u00C2\u00A0reports,\t\r \u00C2\u00A0excluding\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0maintain\t\r \u00C2\u00A0privacy.\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0were\t\r \u00C2\u00A0separated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0Predictive\t\r \u00C2\u00A0or\t\r \u00C2\u00A0Diagnostic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0and\t\r \u00C2\u00A0each\t\r \u00C2\u00A0type\t\r \u00C2\u00A0of\t\r \u00C2\u00A0test\t\r \u00C2\u00A0was\t\r \u00C2\u00A0separated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0year.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Throughout\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0process,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0resource\t\r \u00C2\u00A0director\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Society\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0(HSC)\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0guidance\t\r \u00C2\u00A0in\t\r \u00C2\u00A0confirming\t\r \u00C2\u00A0and\t\r \u00C2\u00A0updating\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0database.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0known\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0deceased\t\r \u00C2\u00A0but\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0as\t\r \u00C2\u00A0such\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0file\t\r \u00C2\u00A0were\t\r \u00C2\u00A0updated,\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0was\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0moved\t\r \u00C2\u00A0locations\t\r \u00C2\u00A0and\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0were\t\r \u00C2\u00A0analyzed\t\r \u00C2\u00A0and\t\r \u00C2\u00A0updated.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0coverage\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0and\t\r \u00C2\u00A0receive\t\r \u00C2\u00A0information\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0records\t\r \u00C2\u00A0outside\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH,\t\r \u00C2\u00A0short\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0were\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0following\t\r \u00C2\u00A0physicians:\t\r \u00C2\u00A01)\t\r \u00C2\u00A0every\t\r \u00C2\u00A0neurologist\t\r \u00C2\u00A0practicing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A02)\t\r \u00C2\u00A0every\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0who\t\r \u00C2\u00A0has\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0for\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0and\t\r \u00C2\u00A03)\t\r \u00C2\u00A0every\t\r \u00C2\u00A0GP\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0that\t\r \u00C2\u00A0serves\t\r \u00C2\u00A0an\t\r \u00C2\u00A0area\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0not\t\r \u00C2\u00A0covered\t\r \u00C2\u00A0by\t\r \u00C2\u00A0either\t\r \u00C2\u00A01)\t\r \u00C2\u00A0or\t\r \u00C2\u00A02).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Physicians\t\r \u00C2\u00A0were\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0if\t\r \u00C2\u00A0they\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0care\t\r \u00C2\u00A0for\t\r \u00C2\u00A0any\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0if\t\r \u00C2\u00A0so\t\r \u00C2\u00A0how\t\r \u00C2\u00A0many.\t\r \u00C2\u00A0\t\r \u00C2\u00A0They\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0to\t\r \u00C2\u00A0list\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0undergone\t\r 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\u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0had\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0via\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0counted\t\r \u00C2\u00A0again.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0list\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0is\t\r \u00C2\u00A0listed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A04.\t\r \u00C2\u00A0\t\r \u00C2\u00A0To\t\r \u00C2\u00A0encourage\t\r \u00C2\u00A0responses,\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0who\t\r \u00C2\u00A0replied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0questionnaire\t\r \u00C2\u00A0were\t\r \u00C2\u00A0entered\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0draw\t\r \u00C2\u00A0to\t\r \u00C2\u00A0win\t\r \u00C2\u00A0an\t\r \u00C2\u00A0iPad2.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition\t\r \u00C2\u00A0to\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires,\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0were\t\r \u00C2\u00A0distributed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0families\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A02011,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HSC\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u0098Family\t\r \u00C2\u00A0Day\u00E2\u0080\u0099\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0attended\t\r \u00C2\u00A0by\t\r \u00C2\u00A0over\t\r \u00C2\u00A0100\t\r \u00C2\u00A0individuals.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0introductory\t\r \u00C2\u00A0presentation\t\r \u00C2\u00A0and\t\r \u00C2\u00A0information\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 19\t\r \u00C2\u00A0 booth\t\r \u00C2\u00A0describing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study\t\r \u00C2\u00A0was\t\r \u00C2\u00A0set\t\r \u00C2\u00A0up.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Surveys\t\r \u00C2\u00A0were\t\r \u00C2\u00A0made\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0those\t\r \u00C2\u00A0members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0interested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0participating.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Surveys\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0participants\t\r \u00C2\u00A0to\t\r \u00C2\u00A0list\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0living\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0to\t\r \u00C2\u00A0their\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0who\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0exhibit\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptoms,\t\r \u00C2\u00A0in\t\r \u00C2\u00A0which\t\r \u00C2\u00A0city,\t\r \u00C2\u00A0who\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0GP\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0neurologist\t\r \u00C2\u00A0is\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0they\t\r \u00C2\u00A0are\t\r \u00C2\u00A0known\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0visited\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A04).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0cover\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0further\t\r \u00C2\u00A0and\t\r \u00C2\u00A0ascertain\t\r \u00C2\u00A0elderly\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0may\t\r \u00C2\u00A0not\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH,\t\r \u00C2\u00A0long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0were\t\r \u00C2\u00A0contacted.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0managers\t\r \u00C2\u00A0at\t\r \u00C2\u00A0these\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0were\t\r \u00C2\u00A0visited,\t\r \u00C2\u00A0emailed\t\r \u00C2\u00A0or\t\r \u00C2\u00A0phoned\t\r \u00C2\u00A0and\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0if\t\r \u00C2\u00A0they\t\r \u00C2\u00A0had\t\r \u00C2\u00A0any\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0diagnosis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0home\t\r \u00C2\u00A0and\t\r \u00C2\u00A0if\t\r \u00C2\u00A0so,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residents.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0they\t\r \u00C2\u00A0were\t\r \u00C2\u00A0asked\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0name\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0resident\u00E2\u0080\u0099s\t\r \u00C2\u00A0referring\t\r \u00C2\u00A0doctor\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0if\t\r \u00C2\u00A0the\t\r \u00C2\u00A0resident\t\r \u00C2\u00A0had\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A049\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0were\t\r \u00C2\u00A0contacted\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A03).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0contacted\t\r \u00C2\u00A0as\t\r \u00C2\u00A0Evergreen\t\r \u00C2\u00A0hamlets\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Surrey\t\r \u00C2\u00A0is\t\r \u00C2\u00A0advertised\t\r \u00C2\u00A0as\t\r \u00C2\u00A0specializing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0(Evergreen\t\r \u00C2\u00A0Hamlets,\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0area\t\r \u00C2\u00A0requiring\t\r \u00C2\u00A0living\t\r \u00C2\u00A0assistance\t\r \u00C2\u00A0are\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0this\t\r \u00C2\u00A0home\t\r \u00C2\u00A0(V.\t\r \u00C2\u00A0Jojin,\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0communication,\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0contacted\t\r \u00C2\u00A0either.\t\r \u00C2\u00A0\t\r \u00C2\u00A0We\t\r \u00C2\u00A0found\t\r \u00C2\u00A0a\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0(non-\u00C2\u00AD\u00E2\u0080\u0090clinic)\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0on\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0include\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Questions\t\r \u00C2\u00A0could\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0formulated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0staff\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0ensure\t\r \u00C2\u00A0their\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0already\t\r \u00C2\u00A0counted.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0International\t\r \u00C2\u00A0Classification\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0(ICD)\t\r \u00C2\u00A0code\t\r \u00C2\u00A0number\t\r \u00C2\u00A0333.0,\t\r \u00C2\u00A0covers\t\r \u00C2\u00A0systemic\t\r \u00C2\u00A0atrophies\t\r \u00C2\u00A0primarily\t\r \u00C2\u00A0affecting\t\r \u00C2\u00A0the\t\r \u00C2\u00A0central\t\r \u00C2\u00A0nervous\t\r \u00C2\u00A0system.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0has\t\r \u00C2\u00A0its\t\r \u00C2\u00A0own\t\r \u00C2\u00A0sub-\u00C2\u00AD\u00E2\u0080\u0090category\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0code,\t\r \u00C2\u00A0under\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0(British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics,\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0death\t\r \u00C2\u00A0occurs,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0corresponding\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0is\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0account\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death.\t\r \u00C2\u00A0\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0publishes\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0describing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0causes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r \u00C2\u00A0under\t\r \u00C2\u00A0each\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0code.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition,\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0maintains\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0for\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0deaths\t\r \u00C2\u00A0under\t\r \u00C2\u00A0each\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0category\t\r \u00C2\u00A0i.e.\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.0\t\r \u00C2\u00A0is\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Stats\t\r \u00C2\u00A0Can\t\r \u00C2\u00A0but\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0is\t\r \u00C2\u00A0not.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0acquire\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 20\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A04.\t\r \u00C2\u00A0Chronology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0 individuals\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0under\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year,\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0were\t\r \u00C2\u00A0reviewed.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Annual\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0from\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902009\t\r \u00C2\u00A0are\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Chart review (1) UBC HD clinic & (2) VGH medical genetics Review of HD genetic tests (4) CMMT research lab, (5) C&W DNA diagnostic lab BC physician records (6) Questionnaires to neurologists, GPs and DNA diagnostic lab referring doctors HSC (3) BC resource director HD family community in BC (7) Surveys to volunteer participants BC nursing home review (8) Personal visits, email and telephone communication ICD code analysis (9) BC Vital Statistics annual reports reviewed \t\r \u00C2\u00A0 21\t\r \u00C2\u00A0 Maximizing\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0numbers:\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0 risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0maximize\t\r \u00C2\u00A0the\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0our\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0scale\t\r \u00C2\u00A0was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0all\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0are\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0never\t\r \u00C2\u00A0themselves\t\r \u00C2\u00A0been\t\r \u00C2\u00A0seen\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics\t\r \u00C2\u00A0but\t\r \u00C2\u00A0are\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0rare\t\r \u00C2\u00A0that\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence,\t\r \u00C2\u00A0full\t\r \u00C2\u00A0name,\t\r \u00C2\u00A0birthdate\t\r \u00C2\u00A0or\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0is\t\r \u00C2\u00A0included\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigree,\t\r 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\u00C2\u00A0contributed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0resulting\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0score:\t\r \u00C2\u00A01)\t\r \u00C2\u00A0the\t\r \u00C2\u00A0likelihood\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0is\t\r \u00C2\u00A0still\t\r \u00C2\u00A0alive\t\r \u00C2\u00A0and\t\r \u00C2\u00A02)\t\r \u00C2\u00A0the\t\r \u00C2\u00A0likelihood\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0is\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD:\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Certainty\t\r \u00C2\u00A0scores:\t\r \u00C2\u00A01) The\t\r \u00C2\u00A0likelihood\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0still\t\r \u00C2\u00A0alive:\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A00:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pedigree,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0ascertained,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0\u00E2\u0089\u00A520\t\r \u00C2\u00A0years\t\r \u00C2\u00A0ago\t\r \u00C2\u00A0(before,\t\r \u00C2\u00A0or\t\r \u00C2\u00A0in,\t\r \u00C2\u00A01992)\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A01:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pedigree,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0ascertained,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0between\t\r \u00C2\u00A010\t\r \u00C2\u00A0and\t\r \u00C2\u00A020\t\r \u00C2\u00A0years\t\r \u00C2\u00A0ago\t\r \u00C2\u00A0(1993-\u00C2\u00AD\u00E2\u0080\u00902001)\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A02:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pedigree,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0ascertained,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0\u00E2\u0089\u00A410\t\r \u00C2\u00A0years\t\r \u00C2\u00A0ago\t\r \u00C2\u00A0(before,\t\r \u00C2\u00A0or\t\r \u00C2\u00A0in,\t\r \u00C2\u00A02002)\t\r \u00C2\u00A02) The\t\r \u00C2\u00A0likelihood\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD:\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A00:\t\r \u00C2\u00A0no\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0information\t\r \u00C2\u00A0or\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0information\t\r \u00C2\u00A0was\t\r \u00C2\u00A0available\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member(s)\t\r \u00C2\u00A0(seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A01:\t\r \u00C2\u00A0\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member(s)\t\r \u00C2\u00A0(seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0were\t\r \u00C2\u00A0clinically\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 22\t\r \u00C2\u00A0 Score\t\r \u00C2\u00A0of\t\r \u00C2\u00A02:\t\r \u00C2\u00A0at\t\r \u00C2\u00A0least\t\r \u00C2\u00A0one\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member\t\r \u00C2\u00A0(seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0has\t\r \u00C2\u00A0tested\t\r \u00C2\u00A0gene\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Defining\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures:\t\r \u00C2\u00A0 \u00E2\u0080\u009CLow\u00E2\u0080\u009D:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A00-\u00C2\u00AD\u00E2\u0080\u00901,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0sum\t\r \u00C2\u00A0of\t\r \u00C2\u00A01)\t\r \u00C2\u00A0and\t\r \u00C2\u00A02)\t\r \u00C2\u00A0above\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \u00E2\u0080\u009CMedium\u00E2\u0080\u009D:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A02,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0sum\t\r \u00C2\u00A0of\t\r \u00C2\u00A01)\t\r \u00C2\u00A0and\t\r \u00C2\u00A02)\t\r \u00C2\u00A0above\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \u00E2\u0080\u009CHigh\u00E2\u0080\u009D:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A0\u00E2\u0089\u00A53,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0sum\t\r \u00C2\u00A0of\t\r \u00C2\u00A01)\t\r \u00C2\u00A0and\t\r \u00C2\u00A02)\t\r \u00C2\u00A0above\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0is\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0process\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertaining\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0from\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0one\t\r \u00C2\u00A0source.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patient\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0information\t\r \u00C2\u00A0is\t\r \u00C2\u00A0confidential\t\r \u00C2\u00A0and\t\r \u00C2\u00A0as\t\r \u00C2\u00A0such,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0identity\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0all\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources,\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0exception\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0review\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0inability\t\r \u00C2\u00A0to\t\r \u00C2\u00A0directly\t\r \u00C2\u00A0crosscheck\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0between\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0causes\t\r \u00C2\u00A0limitations\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0feasibility\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0data\t\r \u00C2\u00A0(Levy\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Feingold,\t\r \u00C2\u00A02000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0would\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0prevalent\t\r \u00C2\u00A0case\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0counted\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0once,\t\r \u00C2\u00A0resulting\t\r \u00C2\u00A0in\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Assuming\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0when\t\r \u00C2\u00A0it\t\r \u00C2\u00A0does\t\r \u00C2\u00A0not\t\r \u00C2\u00A0exist\t\r \u00C2\u00A0would\t\r \u00C2\u00A0result\t\r \u00C2\u00A0in\t\r \u00C2\u00A0failure\t\r \u00C2\u00A0to\t\r \u00C2\u00A0count\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalent\t\r \u00C2\u00A0case\t\r \u00C2\u00A0and\t\r \u00C2\u00A0therefore\t\r \u00C2\u00A0lead\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0underestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0To\t\r \u00C2\u00A0maximize\t\r \u00C2\u00A0the\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0our\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0values\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0lower,\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0and\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0quantify\t\r \u00C2\u00A0the\t\r 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\u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0one\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0locations\t\r \u00C2\u00A0were\t\r \u00C2\u00A0automatically\t\r \u00C2\u00A0removed\t\r \u00C2\u00A0from\t\r \u00C2\u00A0all\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0not\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0analysis,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0indeed\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0that\t\r \u00C2\u00A0had\t\r \u00C2\u00A0never\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics\t\r \u00C2\u00A0run\t\r \u00C2\u00A0the\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0of\t\r \u00C2\u00A0having\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0counted\t\r \u00C2\u00A0from\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0and\t\r \u00C2\u00A0from\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0privacy\t\r \u00C2\u00A0concerns,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0information\t\r \u00C2\u00A0from\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0or\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0and\t\r \u00C2\u00A0family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responders.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0steps\t\r \u00C2\u00A0were\t\r \u00C2\u00A0required\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0the\t\r \u00C2\u00A0possibility\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0prevalent\t\r \u00C2\u00A0case\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A05).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Defining\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0\t\r \u00C2\u00A0 0:\t\r \u00C2\u00A0An\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0(ORS)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0zero\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0an\t\r \u00C2\u00A0extremely\t\r \u00C2\u00A0low\t\r \u00C2\u00A0chance\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0case\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0i.e.\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0an\t\r \u00C2\u00A0extremely\t\r \u00C2\u00A0low\t\r \u00C2\u00A0chance\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0another\t\r \u00C2\u00A0source.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0become\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A00,\t\r \u00C2\u00A0secondary\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0obtained.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0example,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0or\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0that\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r 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\u00C2\u00A0overlapping\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0were\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contacted\t\r \u00C2\u00A0and\t\r \u00C2\u00A0asked,\t\r \u00C2\u00A0if\t\r \u00C2\u00A0possible,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0these\t\r \u00C2\u00A0same\t\r \u00C2\u00A0pieces\t\r \u00C2\u00A0of\t\r \u00C2\u00A0information.\t\r \u00C2\u00A0If\t\r \u00C2\u00A0the\t\r \u00C2\u00A0information\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0were\t\r \u00C2\u00A0to\t\r \u00C2\u00A0match\t\r \u00C2\u00A0our\t\r \u00C2\u00A0information,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0removed\t\r \u00C2\u00A0from\t\r \u00C2\u00A0all\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0known\t\r \u00C2\u00A0with\t\r \u00C2\u00A0near\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0that\t\r 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\u00C2\u00A0remained\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0range\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0only\t\r \u00C2\u00A0(figure\t\r \u00C2\u00A05).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Step\t\r \u00C2\u00A02:\t\r \u00C2\u00A0Check\t\r \u00C2\u00A0if\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0who\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0the\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0response\t\r \u00C2\u00A0serves\t\r \u00C2\u00A0any\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 25\t\r \u00C2\u00A0 who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigree.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0like\t\r \u00C2\u00A0this\t\r \u00C2\u00A0were\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0found,\t\r \u00C2\u00A0information\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0siblings,\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0children\t\r \u00C2\u00A0and\t\r \u00C2\u00A0initials\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0(if\t\r \u00C2\u00A0available)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0recorded.\t\r \u00C2\u00A0These\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0were\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contacted\t\r \u00C2\u00A0and\t\r \u00C2\u00A0asked,\t\r \u00C2\u00A0if\t\r \u00C2\u00A0possible,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0this\t\r \u00C2\u00A0same\t\r \u00C2\u00A0information.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0the\t\r \u00C2\u00A0information\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0were\t\r \u00C2\u00A0to\t\r \u00C2\u00A0match\t\r \u00C2\u00A0our\t\r \u00C2\u00A0information,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0taken\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0all\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0known\t\r \u00C2\u00A0with\t\r \u00C2\u00A0near\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0that\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0counted.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0their\t\r \u00C2\u00A0information\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0match\t\r \u00C2\u00A0ours,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0bumped\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A01\t\r \u00C2\u00A0(not\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member\t\r \u00C2\u00A0does\t\r \u00C2\u00A0not\t\r \u00C2\u00A0necessarily\t\r \u00C2\u00A0indicate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0resident\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient).\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0this\t\r \u00C2\u00A0information\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contact,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0remained\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0only\t\r \u00C2\u00A0(figure\t\r \u00C2\u00A05).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 26\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A05.\t\r \u00C2\u00A0Steps\t\r \u00C2\u00A0taken\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0(O.R.S.)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0 from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires,\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0and\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0by\t\r \u00C2\u00A0ensuring\t\r \u00C2\u00A0they\t\r \u00C2\u00A0 do\t\r \u00C2\u00A0not\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0with\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 27\t\r \u00C2\u00A0 Performing\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0The\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0was\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0into\t\r \u00C2\u00A0a\t\r \u00C2\u00A0lower,\t\r 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\u00C2\u00A0and\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0included\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0of\t\r \u00C2\u00A00,\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A02\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D,\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Clow\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A06.\t\r \u00C2\u00A0Graphical\t\r \u00C2\u00A0representation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0and\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0 measures\t\r \u00C2\u00A0that\t\r \u00C2\u00A0make\t\r \u00C2\u00A0up\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper,\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0and\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 OVERLAP RISK SCORES C ER TA IN TY M EA SU R ES 0 1 2 Low - Medium - High - Patients(included(in(upper%prevalence(range( Patients(included(in(mid(prevalence(range( Patients(included(in(lower%prevalence(range( Certainty(score(total(of( 0-1% Certainty(score(total(of( 2% Certainty(score(total(of( \u00E2\u0089\u00A53% Patients(for(whom(information(could(not(support(or(rule(out(overlap( Information(suggested(this(patient(was(unique(but(secondary(sources(could(not(con;irm(this( Secondary(information(con;irmed(patient(had(not(already(been(counted(( \t\r \u00C2\u00A0 28\t\r 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\u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0also\t\r \u00C2\u00A0included\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D,\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Clow\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00,\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A02.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0only\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0subtracted\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0high\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0were\t\r \u00C2\u00A0those\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0known\t\r \u00C2\u00A0with\t\r \u00C2\u00A0full\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0(i.e.\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0by\t\r \u00C2\u00A0physicians,\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0or\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0already\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition\t\r \u00C2\u00A0to\t\r \u00C2\u00A0these\t\r \u00C2\u00A0definite\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap,\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Clow\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0subtracted\t\r \u00C2\u00A0to\t\r \u00C2\u00A0arrive\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0was\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0range\t\r \u00C2\u00A0minus\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A0those\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0score.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0range\t\r \u00C2\u00A0was\t\r \u00C2\u00A0then\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0in\t\r \u00C2\u00A02012:\t\r \u00C2\u00A04,609,659\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A07).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 29\t\r \u00C2\u00A0 Mortality\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Specific\t\r \u00C2\u00A0parameters\t\r \u00C2\u00A0were\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0account\t\r \u00C2\u00A0for\t\r \u00C2\u00A0potentially\t\r \u00C2\u00A0missed\t\r \u00C2\u00A0mortalities\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0a\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0year\t\r \u00C2\u00A0of\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0subtracted\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0year\t\r \u00C2\u00A0(2012)\t\r \u00C2\u00A0was\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0than\t\r \u00C2\u00A0or\t\r \u00C2\u00A0equal\t\r \u00C2\u00A0to\t\r \u00C2\u00A019\t\r \u00C2\u00A0and\t\r \u00C2\u00A0there\t\r \u00C2\u00A0was\t\r \u00C2\u00A0no\t\r \u00C2\u00A0information\t\r \u00C2\u00A0to\t\r \u00C2\u00A0suggest\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0or\t\r \u00C2\u00A0their\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member\t\r \u00C2\u00A0(whose\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0included\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0on\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0is\t\r \u00C2\u00A0drawn)\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0089\u00A45\t\r \u00C2\u00A0years,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0suspected\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0deceased,\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0and\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0maintained\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0as\t\r \u00C2\u00A0there\t\r \u00C2\u00A0was\t\r \u00C2\u00A0no\t\r \u00C2\u00A0documentation\t\r \u00C2\u00A0available\t\r \u00C2\u00A0to\t\r \u00C2\u00A0ensure\t\r \u00C2\u00A0their\t\r \u00C2\u00A0death.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Nineteen\t\r \u00C2\u00A0years\t\r \u00C2\u00A0was\t\r \u00C2\u00A0chosen\t\r \u00C2\u00A0as\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0the\t\r \u00C2\u00A0approximate\t\r \u00C2\u00A0average\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0duration\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0(Roos\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01993,\t\r \u00C2\u00A0Foroud\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A0ethnicity\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0is\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ethnically\t\r \u00C2\u00A0heterogeneous\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0While\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0is\t\r \u00C2\u00A0predominantly\t\r \u00C2\u00A0Caucasian,\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A025%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0is\t\r \u00C2\u00A0of\t\r \u00C2\u00A0visible\t\r \u00C2\u00A0minority.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Visible\t\r \u00C2\u00A0minorities\t\r \u00C2\u00A0include,\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Asian\t\r \u00C2\u00A0(7%),\t\r \u00C2\u00A0Chinese\t\r \u00C2\u00A0(10%),\t\r \u00C2\u00A0Black\t\r \u00C2\u00A0(0.7%),\t\r \u00C2\u00A0Filipino\t\r \u00C2\u00A0(2%),\t\r \u00C2\u00A0Latin\t\r \u00C2\u00A0American\t\r \u00C2\u00A0(0.7%)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Southeast\t\r \u00C2\u00A0Asian\t\r \u00C2\u00A0(1%)\t\r \u00C2\u00A0(Statistics\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A02006).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0classification\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0groups\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0network\t\r \u00C2\u00A0(EHDN)\t\r \u00C2\u00A0registry5\t\r \u00C2\u00A0is\t\r \u00C2\u00A0slightly\t\r \u00C2\u00A0different\t\r \u00C2\u00A0than\t\r \u00C2\u00A0those\t\r \u00C2\u00A0presented\t\r \u00C2\u00A0above\t\r \u00C2\u00A0by\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Canada.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Categories\t\r \u00C2\u00A0by\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0can\t\r \u00C2\u00A0however\t\r \u00C2\u00A0be\t\r \u00C2\u00A0grouped\t\r \u00C2\u00A0accordingly\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0fit\t\r \u00C2\u00A0with\t\r \u00C2\u00A0and\t\r \u00C2\u00A0be\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0registry.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0review,\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0classification\t\r \u00C2\u00A0was\t\r \u00C2\u00A0followed.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0included:\t\r \u00C2\u00A0Caucasian,\t\r \u00C2\u00A0African-\u00C2\u00AD\u00E2\u0080\u0090North,\t\r \u00C2\u00A0American-\u00C2\u00AD\u00E2\u0080\u0090Latin,\t\r \u00C2\u00A0Asian-\u00C2\u00AD\u00E2\u0080\u0090East,\t\r \u00C2\u00A0Asian-\u00C2\u00AD\u00E2\u0080\u0090West,\t\r \u00C2\u00A0Mixed\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Other\t\r \u00C2\u00A0(EHDN,\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0a\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0ethnicity\t\r \u00C2\u00A0and\t\r \u00C2\u00A0country\t\r \u00C2\u00A0of\t\r \u00C2\u00A0origin\t\r \u00C2\u00A0were\t\r \u00C2\u00A0collected\t\r \u00C2\u00A0wherever\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0process\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A02).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Ethnicity\t\r \u00C2\u00A0was\t\r \u00C2\u00A0also\t\r \u00C2\u00A0requested\t\r \u00C2\u00A0from\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0but\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0requested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0or\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0(questions\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r 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\u00C2\u00A0interested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0taking\t\r \u00C2\u00A0part\t\r \u00C2\u00A0in\t\r \u00C2\u00A0various\t\r \u00C2\u00A0HD-\u00C2\u00AD\u00E2\u0080\u0090associated\t\r \u00C2\u00A0studies.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0maintains\t\r \u00C2\u00A0information\t\r \u00C2\u00A0on\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Europe\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0largest\t\r \u00C2\u00A0database\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0world\t\r \u00C2\u00A0(EHDN,\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 30\t\r \u00C2\u00A0 regarding\t\r \u00C2\u00A0ethnicity\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0excluded\t\r \u00C2\u00A0from\t\r \u00C2\u00A0these\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0complexity\t\r \u00C2\u00A0and\t\r \u00C2\u00A0in\t\r \u00C2\u00A0turn\t\r \u00C2\u00A0maximize\t\r \u00C2\u00A0response\t\r \u00C2\u00A0rate).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0within\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0by\t\r \u00C2\u00A0adding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u0098unknown\u00E2\u0080\u0099\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0expected\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0composed\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0group\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0empirical\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0numbers;\t\r \u00C2\u00A0this\t\r \u00C2\u00A0total\t\r \u00C2\u00A0was\t\r \u00C2\u00A0then\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0group\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0There\t\r \u00C2\u00A0are\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A0938,488\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Asian,\t\r \u00C2\u00A0647,123\t\r \u00C2\u00A0West\t\r \u00C2\u00A0Asian,\t\r \u00C2\u00A031,108\t\r \u00C2\u00A0Latin\t\r \u00C2\u00A0American,\t\r \u00C2\u00A030,410\t\r \u00C2\u00A0North\t\r \u00C2\u00A0African\t\r \u00C2\u00A0and\t\r \u00C2\u00A0219,604\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0Aboriginal\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0in\t\r \u00C2\u00A02012\t\r \u00C2\u00A0(Statistics\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0were\t\r \u00C2\u00A0used\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0denominators\t\r \u00C2\u00A0in\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ethnic-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0figures\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 31\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A08.\t\r \u00C2\u00A0Equation\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0(per\t\r \u00C2\u00A0million/year).\t\r \u00C2\u00A0This\t\r \u00C2\u00A0equation\t\r \u00C2\u00A0 was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0separately\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r \u00C2\u00A0reviewed\t\r \u00C2\u00A0(2000-\u00C2\u00AD\u00E2\u0080\u00902011)\t\r \u00C2\u00A0 Incidence\t\r \u00C2\u00A0\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0summing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0either\t\r \u00C2\u00A0received\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0result\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0had\t\r \u00C2\u00A0experienced\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0of\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r \u00C2\u00A0between\t\r \u00C2\u00A0January\t\r \u00C2\u00A01,\t\r \u00C2\u00A02000\t\r \u00C2\u00A0and\t\r \u00C2\u00A0December\t\r \u00C2\u00A031,\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0number\t\r \u00C2\u00A0was\t\r \u00C2\u00A0divided\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0average\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0respective\t\r \u00C2\u00A0year\t\r \u00C2\u00A0(BC\t\r \u00C2\u00A0Stats,\t\r \u00C2\u00A02012)\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A08).\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r 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\u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0(2009)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0equation\t\r \u00C2\u00A0corrects\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0previously\t\r \u00C2\u00A0static\t\r \u00C2\u00A0denominator\t\r \u00C2\u00A0that\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0used\t\r \u00C2\u00A0to\t\r \u00C2\u00A0represent\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0revised\t\r \u00C2\u00A0denominator\t\r \u00C2\u00A0consists\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0eligible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0but\t\r \u00C2\u00A0only\t\r 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\u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0determined\t\r \u00C2\u00A0using\t\r \u00C2\u00A0two\t\r \u00C2\u00A0methods;\t\r \u00C2\u00A0the\t\r \u00C2\u00A0above\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0used\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0review\t\r \u00C2\u00A0of\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Both\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0were\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0allow\t\r \u00C2\u00A0for\t\r \u00C2\u00A0comparisons\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Uptake\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0time\t\r \u00C2\u00A0period\t\r \u00C2\u00A0of\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02000\t\r \u00C2\u00A0to\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0time\t\r \u00C2\u00A0period\t\r \u00C2\u00A0was\t\r \u00C2\u00A0chosen\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0study\t\r \u00C2\u00A0of\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0reviewed\t\r \u00C2\u00A0the\t\r \u00C2\u00A0period\t\r \u00C2\u00A0from\t\r \u00C2\u00A0January\t\r \u00C2\u00A01,\t\r \u00C2\u00A01987\t\r \u00C2\u00A0to\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02000.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Not\t\r \u00C2\u00A0every\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0report\t\r \u00C2\u00A0was\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review,\t\r \u00C2\u00A0but\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A0each\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 2000 2012 HD1 AR AR AR AR AR 18.8 years HD HD2 AR AR AR AR AR 12 years AR AR AR AR AR HD Individual affected with HD Individual at 50% risk for HD \t\r \u00C2\u00A0 36\t\r \u00C2\u00A0 was\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0this\t\r \u00C2\u00A0reason,\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0test\t\r \u00C2\u00A0participants\t\r \u00C2\u00A0and\t\r \u00C2\u00A0regional-\u00C2\u00AD\u00E2\u0080\u0090uptake\t\r \u00C2\u00A0could\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0determined.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Statistical\t\r \u00C2\u00A0analyses\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0between\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0were\t\r \u00C2\u00A0analyzed\t\r \u00C2\u00A0using\t\r \u00C2\u00A0chi\t\r \u00C2\u00A0square\t\r \u00C2\u00A0analyses.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Standard\t\r \u00C2\u00A0error\t\r \u00C2\u00A0and\t\r \u00C2\u00A095%\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0intervals\t\r \u00C2\u00A0for\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0at-\u00C2\u00AD\u00E2\u0080\u0090risk\t\r \u00C2\u00A0popuation\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0were\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0assumption\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Poisson\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0(Massey\t\r \u00C2\u00A0University,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 37\t\r \u00C2\u00A0 Results\t\r \u00C2\u00A0 Patient\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0results\t\r \u00C2\u00A0 Clinic\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review:\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A01,914\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0comprising\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0775\t\r \u00C2\u00A0families\t\r \u00C2\u00A0were\t\r \u00C2\u00A0reviewed\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Hospital\u00E2\u0080\u0099s\t\r \u00C2\u00A0(VGH)\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0department.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0belong\t\r \u00C2\u00A0to\t\r \u00C2\u00A0anyone\t\r \u00C2\u00A0who\t\r \u00C2\u00A0has\t\r \u00C2\u00A0ever\t\r \u00C2\u00A0been\t\r \u00C2\u00A0seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0one\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0clinics,\t\r \u00C2\u00A0including\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0or\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0related\t\r \u00C2\u00A0to\t\r \u00C2\u00A0or\t\r \u00C2\u00A0connected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0person\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0by\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A0775\t\r \u00C2\u00A0family\t\r \u00C2\u00A0files\t\r \u00C2\u00A0present\t\r \u00C2\u00A0in\t\r \u00C2\u00A0these\t\r \u00C2\u00A0clinics,\t\r \u00C2\u00A0696\t\r \u00C2\u00A0(90%)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0files\t\r \u00C2\u00A0included\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0review.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0410\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0and\t\r \u00C2\u00A099\t\r \u00C2\u00A0from\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0were\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0alive,\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0and\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0day,\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0An\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0127\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0on\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0alive,\t\r \u00C2\u00A0living\t\r \u00C2\u00A0and\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0on\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0day.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0127\t\r \u00C2\u00A0potentially\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A098\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure,\t\r \u00C2\u00A018\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0and\t\r \u00C2\u00A011\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Clow\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A05,\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A05).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Thirteen\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A098\t\r \u00C2\u00A0(13%)\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure,\t\r \u00C2\u00A0in\t\r \u00C2\u00A0addition\t\r \u00C2\u00A0to\t\r \u00C2\u00A0having\t\r \u00C2\u00A0been\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0past\t\r \u00C2\u00A010\t\r \u00C2\u00A0years,\t\r \u00C2\u00A0included\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0their\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigree.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Ten\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A013\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0different\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0from\t\r \u00C2\u00A0where\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0different\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0from\t\r \u00C2\u00A0where\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0and\t\r \u00C2\u00A0families\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responses.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A05\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0assignments.\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0452\t\r \u00C2\u00A0(89%)\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A0360\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A092\t\r \u00C2\u00A0from\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics,\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0via\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A057\t\r \u00C2\u00A0(50\t\r \u00C2\u00A0from\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0and\t\r \u00C2\u00A07\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Victoria)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0clinically\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0presentation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A03).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 38\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A03.\t\r \u00C2\u00A0Numbers\t\r \u00C2\u00A0and\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0and\t\r \u00C2\u00A0without\t\r \u00C2\u00A0a\t\r \u00C2\u00A0 genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0questionnaires:\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0722\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0were\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0to\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC;\t\r \u00C2\u00A0this\t\r \u00C2\u00A0included\t\r \u00C2\u00A0510\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Practitioners\t\r \u00C2\u00A0(GPs),\t\r \u00C2\u00A0132\t\r \u00C2\u00A0neurologists\t\r \u00C2\u00A0and\t\r \u00C2\u00A080\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0blood\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0at\t\r \u00C2\u00A0C&W\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0or\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0testing.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Responses\t\r \u00C2\u00A0were\t\r \u00C2\u00A0received\t\r \u00C2\u00A0from\t\r \u00C2\u00A0174\t\r \u00C2\u00A0GPs\t\r \u00C2\u00A0(34%),\t\r \u00C2\u00A042\t\r \u00C2\u00A0neurologists\t\r \u00C2\u00A0(32%)\t\r \u00C2\u00A0and\t\r \u00C2\u00A027\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab-\u00C2\u00AD\u00E2\u0080\u0090referring\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0(34%).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0was\t\r \u00C2\u00A0243,\t\r \u00C2\u00A0resulting\t\r \u00C2\u00A0in\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overall\t\r \u00C2\u00A0response\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0of\t\r \u00C2\u00A034%\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A04).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0from\t\r \u00C2\u00A019\t\r \u00C2\u00A0GPs\t\r \u00C2\u00A0(46\t\r \u00C2\u00A0patients),\t\r \u00C2\u00A023\t\r \u00C2\u00A0neurologists\t\r \u00C2\u00A0(54\t\r \u00C2\u00A0patients)\t\r \u00C2\u00A0and\t\r \u00C2\u00A011\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab-\u00C2\u00AD\u00E2\u0080\u0090referring\t\r \u00C2\u00A0doctors\t\r \u00C2\u00A0(13\t\r \u00C2\u00A0patients).\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0113\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0an\t\r \u00C2\u00A0additional\t\r \u00C2\u00A048\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0were\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0these\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A03).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0113\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A032\t\r \u00C2\u00A0had\t\r \u00C2\u00A0not\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0genetics\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0(ORS)\t\r \u00C2\u00A0analysis.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A048\t\r \u00C2\u00A0total\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members,\t\r \u00C2\u00A036\t\r \u00C2\u00A0belonged\t\r \u00C2\u00A0to\t\r \u00C2\u00A0families\t\r \u00C2\u00A0of\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0highly\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0these\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0had\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0via\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0definite\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0counted.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A012\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0analysis.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 From\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A081\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A036\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0(117\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0total)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0definite\t\r \u00C2\u00A0overlap,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0had\t\r \u00C2\u00A0responded\t\r \u00C2\u00A0stating\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0had\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0and\t\r \u00C2\u00A0have\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0accounted\t\r \u00C2\u00A0for\t\r \u00C2\u00A0via\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Forty-\u00C2\u00AD\u00E2\u0080\u0090four\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0(including\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0therefore\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0scores.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Nineteen\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0 Ascertainment\t\r \u00C2\u00A0source\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0 patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0 patients\t\r \u00C2\u00A0without\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 Percentage\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0 with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0 452\t\r \u00C2\u00A0 57\t\r \u00C2\u00A0 89%\t\r \u00C2\u00A0Physician\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 17\t\r \u00C2\u00A0 47%\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0 469\t\r \u00C2\u00A0 74\t\r \u00C2\u00A0 86%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 39\t\r \u00C2\u00A0 were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02;\t\r \u00C2\u00A04\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0by\t\r \u00C2\u00A0GPs\t\r \u00C2\u00A0and\t\r \u00C2\u00A015,\t\r \u00C2\u00A0by\t\r \u00C2\u00A0neurologists.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A025\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A01.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0justifications\t\r \u00C2\u00A0for\t\r \u00C2\u00A0these\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0assignments\t\r \u00C2\u00A0are\t\r \u00C2\u00A0described\t\r \u00C2\u00A0in\t\r \u00C2\u00A0detail\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A06.\t\r \u00C2\u00A0\t\r \u00C2\u00A0No\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00;\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0were\t\r \u00C2\u00A0either\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contact\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0information\t\r \u00C2\u00A0required\t\r \u00C2\u00A0to\t\r \u00C2\u00A0rule\t\r \u00C2\u00A0out\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0to\t\r \u00C2\u00A0them\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A032\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0surveys,\t\r \u00C2\u00A015\t\r \u00C2\u00A0(47%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0and\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0be\t\r \u00C2\u00A0genetically\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A017\t\r \u00C2\u00A0were\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0presentation\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A04).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Table\t\r \u00C2\u00A04.\t\r \u00C2\u00A0Summary\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0Responded\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0Response\t\r \u00C2\u00A0Rate\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0resident\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0not\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090referred\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0GPs\t\r \u00C2\u00A0 510\t\r \u00C2\u00A0 174\t\r \u00C2\u00A0 34%\t\r \u00C2\u00A0 46\t\r \u00C2\u00A0 17\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0Neurologists\t\r \u00C2\u00A0 132\t\r \u00C2\u00A0 42\t\r \u00C2\u00A0 32%\t\r \u00C2\u00A0 54\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0Diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0referring\t\r \u00C2\u00A0doctors\t\r \u00C2\u00A0 80\t\r \u00C2\u00A0 27\t\r \u00C2\u00A0 34%\t\r \u00C2\u00A0 13\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0 722\t\r \u00C2\u00A0 243\t\r \u00C2\u00A0 34%\t\r \u00C2\u00A0 113\t\r \u00C2\u00A0 48\t\r \u00C2\u00A0 32\t\r \u00C2\u00A0 12\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0and\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0assessment:\t\r \u00C2\u00A0\t\r \u00C2\u00A0Test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0929\t\r \u00C2\u00A0patients;\t\r \u00C2\u00A0295\t\r \u00C2\u00A0were\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0634\t\r \u00C2\u00A0were\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests.\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests,\t\r \u00C2\u00A0107\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0results\t\r \u00C2\u00A0belonged\t\r \u00C2\u00A0to\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0became\t\r \u00C2\u00A0symptomatic\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Surveys:\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A030\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0were\t\r \u00C2\u00A0distributed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0interested\t\r \u00C2\u00A0participants\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A02011\t\r \u00C2\u00A0HDC\t\r \u00C2\u00A0family\t\r \u00C2\u00A0information\t\r \u00C2\u00A0day.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Six\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0(20%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0returned,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0a\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A036\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A01\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A06\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0(reporting\t\r \u00C2\u00A01\t\r \u00C2\u00A0patient)\t\r \u00C2\u00A0stated\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 40\t\r \u00C2\u00A0 that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A05\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0this\t\r \u00C2\u00A0information;\t\r \u00C2\u00A0the\t\r \u00C2\u00A035\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0these\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0underwent\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0analysis.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Twenty-\u00C2\u00AD\u00E2\u0080\u0090nine\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A035\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0definite\t\r \u00C2\u00A0overlap,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0they\t\r \u00C2\u00A0were\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0large\t\r \u00C2\u00A0metropolitan\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0very\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0via\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0or\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0survey.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Three\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Two\t\r \u00C2\u00A0of\t\r \u00C2\u00A0them\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Princeton,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0city\t\r \u00C2\u00A0from\t\r \u00C2\u00A0where\t\r \u00C2\u00A0no\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0were\t\r \u00C2\u00A0received\t\r \u00C2\u00A0and\t\r \u00C2\u00A0from\t\r \u00C2\u00A0where\t\r \u00C2\u00A0no\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0families\t\r \u00C2\u00A0with\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0members\t\r \u00C2\u00A0found\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0are\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0live\t\r \u00C2\u00A0and\t\r \u00C2\u00A0one\t\r \u00C2\u00A0resided\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Tsawwassen,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0city\t\r \u00C2\u00A0where\t\r \u00C2\u00A0only\t\r \u00C2\u00A0one\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0via\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Three\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A01,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0all\t\r \u00C2\u00A0three\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Tsawwassen\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A04,\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0assessment:\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A049\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0contacted,\t\r \u00C2\u00A032\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0(65%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0contact\t\r \u00C2\u00A0and\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0the\t\r \u00C2\u00A0requested\t\r \u00C2\u00A0information.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Four\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0(8%)\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0location:\t\r \u00C2\u00A01\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Penticton,\t\r \u00C2\u00A02\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Westbank,\t\r \u00C2\u00A01\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Powell\t\r \u00C2\u00A0River\t\r \u00C2\u00A0and\t\r \u00C2\u00A014\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Surrey\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A014\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Surrey\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Powell\t\r \u00C2\u00A0River\t\r \u00C2\u00A0had\t\r \u00C2\u00A0already\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0removed\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0analysis.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Penticton\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Westbank\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0staff\t\r \u00C2\u00A0was\t\r \u00C2\u00A0unable\t\r \u00C2\u00A0to\t\r \u00C2\u00A0answer\t\r \u00C2\u00A0the\t\r \u00C2\u00A0necessary\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0required\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0totals:\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 For\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees,\t\r \u00C2\u00A0resident\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0were\t\r \u00C2\u00A0used\t\r \u00C2\u00A0to\t\r \u00C2\u00A0formulate\t\r \u00C2\u00A0questions.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Questions\t\r \u00C2\u00A0maintained\t\r \u00C2\u00A0the\t\r \u00C2\u00A0privacy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0used\t\r \u00C2\u00A0to\t\r \u00C2\u00A0follow-\u00C2\u00AD\u00E2\u0080\u0090up\t\r \u00C2\u00A0with\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaire,\t\r \u00C2\u00A0family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0and\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0best\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0attempt\t\r \u00C2\u00A0to\t\r \u00C2\u00A0rule\t\r \u00C2\u00A0out\t\r \u00C2\u00A0overlap.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires,\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0and\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0are\t\r \u00C2\u00A0listed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0column\t\r \u00C2\u00A08\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A06\t\r \u00C2\u00A0and\t\r \u00C2\u00A0each\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0case\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0is\t\r \u00C2\u00A0described\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0footnotes\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 41\t\r \u00C2\u00A0 labeled\t\r \u00C2\u00A0in\t\r \u00C2\u00A0column\t\r \u00C2\u00A09.\t\r \u00C2\u00A0\t\r \u00C2\u00A0No\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00\t\r \u00C2\u00A0(patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0analysis).\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0follow-\u00C2\u00AD\u00E2\u0080\u0090up\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0either\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0the\t\r \u00C2\u00A0existence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0or\t\r \u00C2\u00A0could\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0answered\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A06).\t\r \u00C2\u00A0Twenty-\u00C2\u00AD\u00E2\u0080\u0090eight\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A01.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Three\t\r \u00C2\u00A0were\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0and\t\r \u00C2\u00A025\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires.\t\r \u00C2\u00A0Twenty-\u00C2\u00AD\u00E2\u0080\u0090five\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Three\t\r \u00C2\u00A0from\t\r \u00C2\u00A0long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0homes,\t\r \u00C2\u00A03\t\r \u00C2\u00A0from\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0and\t\r \u00C2\u00A019\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0questionnaires.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Mortality\t\r \u00C2\u00A0estimates:\t\r \u00C2\u00A0\t\r \u00C2\u00A0 A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A030\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0presumed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0deceased\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0and\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0between\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0year\t\r \u00C2\u00A0(2012)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year,\t\r \u00C2\u00A0on\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0began,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0\u00E2\u0089\u00A519\t\r \u00C2\u00A0for\t\r \u00C2\u00A0these\t\r \u00C2\u00A030\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Additionally,\t\r \u00C2\u00A0there\t\r \u00C2\u00A0was\t\r \u00C2\u00A0no\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0nor\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0of\t\r \u00C2\u00A0their\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member(s)\t\r \u00C2\u00A0to\t\r \u00C2\u00A0suggest\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0still\t\r \u00C2\u00A0living.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0The\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0included\t\r \u00C2\u00A0all\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0all\t\r \u00C2\u00A0ORS\u00E2\u0080\u0099s.\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0added\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0(509),\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0included\t\r \u00C2\u00A0was\t\r \u00C2\u00A0689,\t\r \u00C2\u00A0resulting\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A014.9/100,000\t\r \u00C2\u00A0(1/6,711)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A013.8-\u00C2\u00AD\u00E2\u0080\u009016.0).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0included\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009Cmedium\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0ORS\u00E2\u0080\u0099s\t\r \u00C2\u00A0of\t\r \u00C2\u00A00\t\r \u00C2\u00A0and\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A0excluded\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0presumed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0deceased\t\r \u00C2\u00A0(30\t\r \u00C2\u00A0patients).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0included\t\r \u00C2\u00A0was\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0623,\t\r \u00C2\u00A0resulting\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A013.5/100,000\t\r \u00C2\u00A0(1/7,407)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A012.4-\u00C2\u00AD\u00E2\u0080\u009014.6).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0included\t\r \u00C2\u00A0only\t\r \u00C2\u00A0\u00E2\u0080\u009Chigh\u00E2\u0080\u009D\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0and\t\r \u00C2\u00A0ORS\u00E2\u0080\u0099s\t\r \u00C2\u00A0of\t\r \u00C2\u00A00\t\r \u00C2\u00A0and\t\r \u00C2\u00A0also\t\r \u00C2\u00A0excluded\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0presumed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0deceased\t\r \u00C2\u00A0(30),\t\r \u00C2\u00A0totaling\t\r \u00C2\u00A0577\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A012.5/100,000\t\r \u00C2\u00A0(1/8,000)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A011.5-\u00C2\u00AD\u00E2\u0080\u009013.5)\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A05,\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A013).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0for\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0from\t\r \u00C2\u00A012.5-\u00C2\u00AD\u00E2\u0080\u009014.9/100,000\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A011.5-\u00C2\u00AD\u00E2\u0080\u009016.0;\t\r \u00C2\u00A01/8,696-\u00C2\u00AD\u00E2\u0080\u00901/6,250)\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 42\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A05.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0source\t\r \u00C2\u00A0under\t\r \u00C2\u00A0each\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0 measure\t\r \u00C2\u00A0or\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0is\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0below.\t\r \u00C2\u00A0Each\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper,\t\r \u00C2\u00A0mid\t\r \u00C2\u00A0and\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0 prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0calculations\t\r \u00C2\u00A0is\t\r \u00C2\u00A0broken\t\r \u00C2\u00A0down\t\r \u00C2\u00A0Ascertainment\t\r \u00C2\u00A0source\t\r \u00C2\u00A0 Certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0 Definite\t\r \u00C2\u00A0Overlap\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0Low\t\r \u00C2\u00A0 Medium\t\r \u00C2\u00A0 High\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0 18\t\r \u00C2\u00A0 98\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 127\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0 27\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 383\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 410\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 96\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 99\t\r \u00C2\u00A0Long\t\r \u00C2\u00A0term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0home\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 18\t\r \u00C2\u00A0Family\t\r \u00C2\u00A0surveys\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 30\t\r \u00C2\u00A0 36\t\r \u00C2\u00A0Physician\t\r \u00C2\u00A0questionnaires\t\r \u00C2\u00A0 19\t\r \u00C2\u00A0 25\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 117\t\r \u00C2\u00A0 161\t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0 Upper\t\r \u00C2\u00A0 Mid\t\r \u00C2\u00A0 Lower\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0included\t\r \u00C2\u00A0 0+1+2\t\r \u00C2\u00A0 0+1\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0Certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0included\t\r \u00C2\u00A0 High\t\r \u00C2\u00A0+\t\r \u00C2\u00A0Medium\t\r \u00C2\u00A0+\t\r \u00C2\u00A0Low\t\r \u00C2\u00A0 High\t\r \u00C2\u00A0+\t\r \u00C2\u00A0Medium\t\r \u00C2\u00A0 High\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0 689\t\r \u00C2\u00A0 623\t\r \u00C2\u00A0 577\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0 14.9\t\r \u00C2\u00A0 13.5\t\r \u00C2\u00A0 12.5\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0(1/X)\t\r \u00C2\u00A0 1/6,711\t\r \u00C2\u00A0 1/7,407\t\r \u00C2\u00A0 1/8,000\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 43\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r 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\u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0number\t\r \u00C2\u00A0breakdown\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the:\t\r \u00C2\u00A0a.\t\r \u00C2\u00A0lower,\t\r \u00C2\u00A0b.\t\r \u00C2\u00A0 mid\t\r \u00C2\u00A0and\t\r \u00C2\u00A0c.\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range\t\r \u00C2\u00A0 =\t\r \u00C2\u00A0577\t\r \u00C2\u00A0 =\t\r \u00C2\u00A0623\t\r \u00C2\u00A0 =\t\r \u00C2\u00A0689\t\r \u00C2\u00A0 162$52$127$ 348$ Clinic$charts$509$Surveys$+$$nursing$homes$215$Clinic$pedigrees$127$ 162$ 28$ 24$92$ 317$ Clinic$charts$479$ Clinic$pedigrees$116$ Clinic&charts&479& Clinic&pedigrees&98& 162&53&45& 317& \t\r \u00C2\u00A0 44\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0composition\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population\t\r \u00C2\u00A0The\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0revealed\t\r \u00C2\u00A0490\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0Caucasian,\t\r \u00C2\u00A0one\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0African-\u00C2\u00AD\u00E2\u0080\u0090North,\t\r \u00C2\u00A0one\t\r \u00C2\u00A0American-\u00C2\u00AD\u00E2\u0080\u0090Latin,\t\r \u00C2\u00A0six\t\r \u00C2\u00A0Asian-\u00C2\u00AD\u00E2\u0080\u0090East,\t\r \u00C2\u00A016\t\r \u00C2\u00A0Asian-\u00C2\u00AD\u00E2\u0080\u0090West\t\r \u00C2\u00A0and\t\r \u00C2\u00A0one\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0Aboriginal\t\r \u00C2\u00A0(the\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0Aboriginal\t\r \u00C2\u00A0belongs\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0\u00E2\u0080\u0098Other\u00E2\u0080\u0099\t\r \u00C2\u00A0category).\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition,\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A0113\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0were\t\r \u00C2\u00A0of\t\r \u00C2\u00A0unknown\t\r \u00C2\u00A0ethnicity.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0used\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0comprises\t\r \u00C2\u00A0the\t\r \u00C2\u00A0average\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0three\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0\t\r \u00C2\u00A0After\t\r \u00C2\u00A0extrapolating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0empirical\t\r \u00C2\u00A0data\t\r \u00C2\u00A0to\t\r \u00C2\u00A0include\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u0098Unknown\u00E2\u0080\u0099\t\r \u00C2\u00A0category,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Caucasian\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0became\t\r \u00C2\u00A0597,\t\r \u00C2\u00A0total\t\r \u00C2\u00A0North\t\r \u00C2\u00A0African,\t\r \u00C2\u00A01;\t\r \u00C2\u00A0Latin\t\r \u00C2\u00A0American,\t\r \u00C2\u00A01;\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0Aboriginal,\t\r \u00C2\u00A01;\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Asian,\t\r \u00C2\u00A07\t\r \u00C2\u00A0and\t\r \u00C2\u00A0West\t\r \u00C2\u00A0Asian,\t\r \u00C2\u00A020.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A07\t\r \u00C2\u00A0lists\t\r \u00C2\u00A0the\t\r \u00C2\u00A0countries\t\r \u00C2\u00A0of\t\r \u00C2\u00A0origin\t\r \u00C2\u00A0and\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0country\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0each\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0category.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A014\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0group\t\r \u00C2\u00A0with\t\r \u00C2\u00A0respect\t\r \u00C2\u00A0to\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0group\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A014.\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0separated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0ethnicity.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0pie\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0relative\t\r \u00C2\u00A0 proportions\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0Breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u0098Other\u00E2\u0080\u0099\t\r \u00C2\u00A0 category\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0population\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0listed\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group.\t\r \u00C2\u00A0 Ethnic-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0are\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0in\t\r \u00C2\u00A0column\t\r \u00C2\u00A04\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0table.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 490$(78%)$ 25$(~4%)$ 113$(18%)$ Caucasian$ Other$ Unknown$ Origin&(ethnicity)& & #&of& pa3ents& & & Percent&of& pa3ent& popula3on& & Ethnic9 specific& prevalence& (#/100,000)$ $ Ethnic9 specific& prevalence& (1/X)$ $ Caucasian&(countries$ listed$in$Appendix$7)& 597$ 95%$ 17.2& 1/5,815$ Tanzania& (AfricanINorth)$ 1$ 0.2%$ 4.14& 1/24,155$ Mexico$$ (AmericanILaNn)$ 1$ 0.2%$ 4.05& 1/24,691$ Philippines,&Hong& Kong&(AsianIEast)$ 7$ 1.2%$ 0.78& 1/128,205$ Pakistan,& Afghanistan,&India,& Iran,&Iraq&(AsianI West)$ 20$ 3.0%$ 3.01& 1/33,222$ Aboriginal9Canada&& (Other)$ 1$ 0.2%$ 0.57& 1/175,439$ Number of patients in BC (% of total) \t\r \u00C2\u00A0 45\t\r \u00C2\u00A0 Geographic\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0Patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A096\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A06\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0source\t\r \u00C2\u00A0and\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0city.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A0231-\u00C2\u00AD\u00E2\u0080\u0090245\t\r \u00C2\u00A0(48%)\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0range\t\r \u00C2\u00A0reflects\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0and\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0respectively.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0average\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A022.1/100,000\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A019.3-\u00C2\u00AD\u00E2\u0080\u009024.9;\t\r \u00C2\u00A01/5,181-\u00C2\u00AD\u00E2\u0080\u00901/4,016).\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0248-\u00C2\u00AD\u00E2\u0080\u0090264\t\r \u00C2\u00A0(52%)\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0average\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0at\t\r \u00C2\u00A07.2/100,000\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A06.4-\u00C2\u00AD\u00E2\u0080\u00908.1;\t\r \u00C2\u00A015,625-\u00C2\u00AD\u00E2\u0080\u00901/12,346),\t\r \u00C2\u00A0whereas\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0was\t\r \u00C2\u00A09.3/100,000.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0(P<0.0001,\t\r \u00C2\u00A0t=10.2)\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A015).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0ratio\t\r \u00C2\u00A0of\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D:\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A01:3.1.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A015.\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0separated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Rural=\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A02\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0away\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car;\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Urban=\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0 Rural Urban Vancouver Victoria Prince George 231-245 patients (48%) ~22.1/100,000 264-248 patients (52%) ~7.2/100,000 \t\r \u00C2\u00A0 46\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A016\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0a\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0each\t\r \u00C2\u00A0provincial\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0range\t\r \u00C2\u00A0includes\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0high\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0range\t\r \u00C2\u00A0includes\t\r \u00C2\u00A0all\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0(ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A00,\t\r \u00C2\u00A01\t\r \u00C2\u00A0and\t\r \u00C2\u00A02\t\r \u00C2\u00A0and\t\r \u00C2\u00A0high,\t\r \u00C2\u00A0medium\t\r \u00C2\u00A0and\t\r \u00C2\u00A0low\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0regional\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0range\t\r \u00C2\u00A0from\t\r \u00C2\u00A08.9/100,000\t\r \u00C2\u00A0(1/11,236),\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Fraser\t\r \u00C2\u00A0health\t\r \u00C2\u00A0authority,\t\r \u00C2\u00A0to\t\r \u00C2\u00A020.4/100,000\t\r \u00C2\u00A0(1/4,902)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Authority\t\r \u00C2\u00A0however\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0in\t\r \u00C2\u00A0all\t\r \u00C2\u00A0health\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0do\t\r \u00C2\u00A0not\t\r \u00C2\u00A0differ\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0from\t\r \u00C2\u00A0one\t\r \u00C2\u00A0another\t\r \u00C2\u00A0(ANOVA,\t\r \u00C2\u00A0F=1.83,\t\r \u00C2\u00A0P=0.26)\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A016).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A016.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0each\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbian\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region\t\r \u00C2\u00A0 based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0and\t\r \u00C2\u00A0upper\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0range.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0colour\t\r \u00C2\u00A0key\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0 (/100,000)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0respective\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 32 \u00E2\u0080\u0093 47 patients 84 \u00E2\u0080\u0093 124 patients 148 \u00E2\u0080\u0093 199 patients 125 \u00E2\u0080\u0093 162 patients 115 \u00E2\u0080\u0093 140 patients 10.3 \u00E2\u0080\u0093 15.1 Northern health 11.1 \u00E2\u0080\u0093 13.5 Vancouver Coastal health 10.9 \u00E2\u0080\u0093 16.1 Interior health 15.7 \u00E2\u0080\u0093 20.4 Vancouver Island health 8.9 \u00E2\u0080\u0093 12.0 Fraser health Regional Prevalence (#/100,000) Health region \t\r \u00C2\u00A0 47\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0350\t\r \u00C2\u00A0incident\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0were\t\r \u00C2\u00A0counted\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0these,\t\r \u00C2\u00A0235\t\r \u00C2\u00A0(67%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0recipients,\t\r \u00C2\u00A085\t\r \u00C2\u00A0(24%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0recipients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0experienced\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period,\t\r \u00C2\u00A0and\t\r \u00C2\u00A030\t\r \u00C2\u00A0(9%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0not\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0or\t\r \u00C2\u00A0for\t\r \u00C2\u00A0whom\t\r \u00C2\u00A0results\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0but\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0experienced\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0yearly\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011\t\r \u00C2\u00A0ranged\t\r \u00C2\u00A0from\t\r \u00C2\u00A03.7\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/\t\r \u00C2\u00A0year\t\r \u00C2\u00A0in\t\r \u00C2\u00A02011\t\r \u00C2\u00A0to\t\r \u00C2\u00A09.1\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0in\t\r \u00C2\u00A02005.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0average\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0was\t\r \u00C2\u00A07.2\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A06.5-\u00C2\u00AD\u00E2\u0080\u00907.9)\t\r \u00C2\u00A0over\t\r \u00C2\u00A0the\t\r \u00C2\u00A0twelve-\u00C2\u00AD\u00E2\u0080\u0090year\t\r \u00C2\u00A0period\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A017,\t\r \u00C2\u00A0Table\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test-\u00C2\u00AD\u00E2\u0080\u0090positive\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0ranged\t\r \u00C2\u00A0from\t\r \u00C2\u00A01.6-\u00C2\u00AD\u00E2\u0080\u00904.8\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0average\t\r \u00C2\u00A0of\t\r \u00C2\u00A03.3\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0over\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ten-\u00C2\u00AD\u00E2\u0080\u0090year\t\r \u00C2\u00A0period\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A06).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 8.8\t\r \u00C2\u00A0 8.5\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 9.1\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 8.6\t\r \u00C2\u00A0 7.8\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0 3.7\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 12\t\r \u00C2\u00A0 14\t\r \u00C2\u00A0 2000\t\r \u00C2\u00A0 2001\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 2003\t\r \u00C2\u00A0 2004\t\r \u00C2\u00A0 2005\t\r \u00C2\u00A0 2006\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 2009\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 2011\t\r \u00C2\u00A0 Inciden ce\t\r \u00C2\u00A0(/m illion/y ear)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A017.\t\r \u00C2\u00A0Estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0(/million/year)\t\r \u00C2\u00A0from\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011.\t\r \u00C2\u00A0Error\t\r \u00C2\u00A0 bars\t\r \u00C2\u00A0represent\t\r \u00C2\u00A095%\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0intervals\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 48\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A06.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0showing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0included\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0source,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incident\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0and\t\r \u00C2\u00A0 the\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0(DT)-\u00C2\u00AD\u00E2\u0080\u0090positive\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0year\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902011\t\r \u00C2\u00A0 Year\t\r \u00C2\u00A0 Pop.\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\t\r \u00C2\u00A0 #\t\r \u00C2\u00A0of\t\r \u00C2\u00A0(+)\t\r \u00C2\u00A0DT\t\r \u00C2\u00A0\t\r \u00C2\u00A0 #\t\r \u00C2\u00A0of\t\r \u00C2\u00A0(+)\t\r \u00C2\u00A0PT\t\r \u00C2\u00A0and\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0(2000-\u00C2\u00AD\u00E2\u0080\u00902011)\t\r \u00C2\u00A0 #\t\r \u00C2\u00A0with\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0test\t\r \u00C2\u00A0unavailable\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0#\t\r \u00C2\u00A0incident\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0 Incidence\t\r \u00C2\u00A0(/million/yr.)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI)\t\r \u00C2\u00A0 DT\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0(/million/yr.)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI)\t\r \u00C2\u00A02000\t\r \u00C2\u00A0 4039200\t\r \u00C2\u00A0 19\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 28\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0(4.4-\u00C2\u00AD\u00E2\u0080\u00909.5)\t\r \u00C2\u00A0 3.7\t\r \u00C2\u00A0(2.0-\u00C2\u00AD\u00E2\u0080\u00905.4)\t\r \u00C2\u00A02001\t\r \u00C2\u00A0 4,076,264\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 28\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0(4.3-\u00C2\u00AD\u00E2\u0080\u00909.4)\t\r \u00C2\u00A0 2.2\t\r \u00C2\u00A0(1.1-\u00C2\u00AD\u00E2\u0080\u00903.2)\t\r \u00C2\u00A02002\t\r \u00C2\u00A0 4,098,178\t\r \u00C2\u00A0 24\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 36\t\r \u00C2\u00A0 8.8\t\r \u00C2\u00A0(5.9-\u00C2\u00AD\u00E2\u0080\u009011.7)\t\r \u00C2\u00A0 4.4\t\r \u00C2\u00A0(2.6-\u00C2\u00AD\u00E2\u0080\u00906.2)\t\r \u00C2\u00A02003\t\r \u00C2\u00A0 4,122,396\t\r \u00C2\u00A0 31\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 35\t\r \u00C2\u00A0 8.5\t\r \u00C2\u00A0(5.7-\u00C2\u00AD\u00E2\u0080\u009011.3)\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0(2.2-\u00C2\u00AD\u00E2\u0080\u00904.6)\t\r \u00C2\u00A02004\t\r \u00C2\u00A0 4,155,170\t\r \u00C2\u00A0 23\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 35\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0(5.6-\u00C2\u00AD\u00E2\u0080\u009011.2)\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0(2.7-\u00C2\u00AD\u00E2\u0080\u00906.5)\t\r \u00C2\u00A02005\t\r \u00C2\u00A0 4,196,788\t\r \u00C2\u00A0 28\t\r \u00C2\u00A0 9\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 38\t\r \u00C2\u00A0 9.1\t\r \u00C2\u00A0(6.2-\u00C2\u00AD\u00E2\u0080\u009011.9)\t\r \u00C2\u00A0 4.8\t\r \u00C2\u00A0(3.0-\u00C2\u00AD\u00E2\u0080\u00906.6)\t\r \u00C2\u00A02006\t\r \u00C2\u00A0 4,243,580\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 22\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0(3.0-\u00C2\u00AD\u00E2\u0080\u00907.4)\t\r \u00C2\u00A0 2.4\t\r \u00C2\u00A0(1.2-\u00C2\u00AD\u00E2\u0080\u00903.6)\t\r \u00C2\u00A02007\t\r \u00C2\u00A0 4,309,632\t\r \u00C2\u00A0 21\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 37\t\r \u00C2\u00A0 8.6\t\r \u00C2\u00A0(5.8-\u00C2\u00AD\u00E2\u0080\u009011.4)\t\r \u00C2\u00A0 3.9\t\r \u00C2\u00A0(2.2-\u00C2\u00AD\u00E2\u0080\u00905.6)\t\r \u00C2\u00A02008\t\r \u00C2\u00A0 4,383,860\t\r \u00C2\u00A0 25\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 34\t\r \u00C2\u00A0 7.8\t\r \u00C2\u00A0(5.2-\u00C2\u00AD\u00E2\u0080\u009010.4)\t\r \u00C2\u00A0 2.7\t\r \u00C2\u00A0(1.6-\u00C2\u00AD\u00E2\u0080\u00903.8)\t\r \u00C2\u00A02009\t\r \u00C2\u00A0 4,460,292\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 12\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 32\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0(4.7-\u00C2\u00AD\u00E2\u0080\u00909.7)\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0(0.79-\u00C2\u00AD\u00E2\u0080\u00902.4)\t\r \u00C2\u00A02010\t\r \u00C2\u00A0 4,530,960\t\r \u00C2\u00A0 17\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 25\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0(3.4-\u00C2\u00AD\u00E2\u0080\u00907.7)\t\r \u00C2\u00A0 2.0\t\r \u00C2\u00A0(1.0-\u00C2\u00AD\u00E2\u0080\u00903.0)\t\r \u00C2\u00A02011\t\r \u00C2\u00A0 4,472,600\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 9\t\r \u00C2\u00A0 17\t\r \u00C2\u00A0 3.7\t\r \u00C2\u00A0(1.2-\u00C2\u00AD\u00E2\u0080\u00905.5)\t\r \u00C2\u00A0 1.1\t\r \u00C2\u00A0(0.29-\u00C2\u00AD\u00E2\u0080\u00901.9)\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 235\t\r \u00C2\u00A0 85\t\r \u00C2\u00A0 30\t\r \u00C2\u00A0 350\t\r \u00C2\u00A0 Mean\t\r \u00C2\u00A0 4,257,410\t\r \u00C2\u00A0 20\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 32\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0(6.5-\u00C2\u00AD\u00E2\u0080\u00907.9)\t\r \u00C2\u00A0 3.1\t\r \u00C2\u00A0(1.7-\u00C2\u00AD\u00E2\u0080\u00904.5)\t\r \u00C2\u00A0%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 67%\t\r \u00C2\u00A0 24%\t\r \u00C2\u00A0 9%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 ICD\t\r \u00C2\u00A0codes\t\r \u00C2\u00A0and\t\r \u00C2\u00A0mortality\t\r \u00C2\u00A0rates:\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 BC\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0only\t\r \u00C2\u00A0began\t\r \u00C2\u00A0to\t\r \u00C2\u00A0record\t\r \u00C2\u00A0the\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0breakdown\t\r \u00C2\u00A0for\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0(Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A02000.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0only\t\r \u00C2\u00A0considered\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0categories,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0was\t\r \u00C2\u00A0grouped\t\r \u00C2\u00A0in\t\r \u00C2\u00A0with\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.0.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Annual\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0were\t\r \u00C2\u00A0only\t\r \u00C2\u00A0available\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A02009\t\r \u00C2\u00A0(British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Agency\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0at\t\r \u00C2\u00A0death\t\r \u00C2\u00A0under\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902009\t\r \u00C2\u00A0is\t\r \u00C2\u00A0124\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0highest\t\r \u00C2\u00A0number\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0single\t\r \u00C2\u00A0year\t\r \u00C2\u00A0occurred\t\r \u00C2\u00A0in\t\r \u00C2\u00A02004\t\r \u00C2\u00A0and\t\r \u00C2\u00A0reported\t\r \u00C2\u00A019\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0as\t\r \u00C2\u00A0their\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A07).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0average\t\r \u00C2\u00A0mortality\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0when\t\r \u00C2\u00A0taking\t\r \u00C2\u00A0only\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0codes\t\r \u00C2\u00A0into\t\r \u00C2\u00A0account\t\r \u00C2\u00A0is\t\r \u00C2\u00A02.9\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A01.3-\u00C2\u00AD\u00E2\u0080\u00904.5)\t\r \u00C2\u00A0between\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902009\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0range\t\r \u00C2\u00A0of\t\r \u00C2\u00A01.5-\u00C2\u00AD\u00E2\u0080\u00904.6\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A07).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 49\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A07.\t\r \u00C2\u00A0Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0at\t\r \u00C2\u00A0death\t\r \u00C2\u00A0under\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0code\t\r \u00C2\u00A0333.0\t\r \u00C2\u00A0and\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0 between\t\r \u00C2\u00A02000\t\r \u00C2\u00A0and\t\r \u00C2\u00A02009\t\r \u00C2\u00A0 Year\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0333.0\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0 Mortality\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0 CNS\t\r \u00C2\u00A0atrophies\t\r \u00C2\u00A0 (/million/yr)\t\r \u00C2\u00A0 (95%\t\r \u00C2\u00A0CI)\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0ICD\t\r \u00C2\u00A0333.4\t\r \u00C2\u00A0 recorded\t\r \u00C2\u00A0 Mortality\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0 for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0 (/million/yr)\t\r \u00C2\u00A0 (95%\t\r \u00C2\u00A0CI)\t\r \u00C2\u00A02000\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 2.5\t\r \u00C2\u00A0(1.0-\u00C2\u00AD\u00E2\u0080\u00904.1)\t\r \u00C2\u00A02001\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 12\t\r \u00C2\u00A0 2.9\t\r \u00C2\u00A0(1.3-\u00C2\u00AD\u00E2\u0080\u00904.5)\t\r \u00C2\u00A02002\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0(0.4-\u00C2\u00AD\u00E2\u0080\u00903.0)\t\r \u00C2\u00A02003\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 1.5\t\r \u00C2\u00A0(0.3-\u00C2\u00AD\u00E2\u0080\u00902.7)\t\r \u00C2\u00A02004\t\r \u00C2\u00A0 111.3\t\r \u00C2\u00A0 24.3\t\r \u00C2\u00A0(23.0-\u00C2\u00AD\u00E2\u0080\u009028.8)\t\r \u00C2\u00A0 19\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0(2.5-\u00C2\u00AD\u00E2\u0080\u00906.7)\t\r \u00C2\u00A02005\t\r \u00C2\u00A0 116.8\t\r \u00C2\u00A0 25.6\t\r \u00C2\u00A0(23.7-\u00C2\u00AD\u00E2\u0080\u009030.2)\t\r \u00C2\u00A0 14\t\r \u00C2\u00A0 3.3\t\r \u00C2\u00A0(1.6-\u00C2\u00AD\u00E2\u0080\u00905.0)\t\r \u00C2\u00A02006\t\r \u00C2\u00A0 133.3\t\r \u00C2\u00A0 24.8\t\r \u00C2\u00A0(21.5-\u00C2\u00AD\u00E2\u0080\u009029.0)\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 3.5\t\r \u00C2\u00A0(1.7-\u00C2\u00AD\u00E2\u0080\u00905.3)\t\r \u00C2\u00A0\t\r \u00C2\u00A0(\t\r \u00C2\u00A02007\t\r \u00C2\u00A0 122.6\t\r \u00C2\u00A0 26.8\t\r \u00C2\u00A0(22.1-\u00C2\u00AD\u00E2\u0080\u009031.5)\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 2.3\t\r \u00C2\u00A0(0.9-\u00C2\u00AD\u00E2\u0080\u00903.7)\t\r \u00C2\u00A02008\t\r \u00C2\u00A0 126.2\t\r \u00C2\u00A0 27.6\t\r \u00C2\u00A0(22.8-\u00C2\u00AD\u00E2\u0080\u009032.4)\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 3.6\t\r \u00C2\u00A0(1.8-\u00C2\u00AD\u00E2\u0080\u00905.4)\t\r \u00C2\u00A02009\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0(1.7-\u00C2\u00AD\u00E2\u0080\u00905.1)\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0 610.2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 124\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Average\t\r \u00C2\u00A0 122.0\t\r \u00C2\u00A0 25.8\t\r \u00C2\u00A0 12.4\t\r \u00C2\u00A0 2.9\t\r \u00C2\u00A0(1.3-\u00C2\u00AD\u00E2\u0080\u00904.5)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 50\t\r \u00C2\u00A0 Minimum\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0 A-\u00C2\u00AD\u00E2\u0080\u0090priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0categories:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A02,252\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0signifying\t\r \u00C2\u00A0that\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A049.2/100,000\t\r \u00C2\u00A0(1/2,047)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0are\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A02,045\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0therefore\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A044.7/100,000\t\r \u00C2\u00A0(1/2,254)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0are\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0Taken\t\r \u00C2\u00A0together,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A04,297\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0(94.0/100,000\t\r \u00C2\u00A0or\t\r \u00C2\u00A01/1,073)\t\r \u00C2\u00A0are\t\r \u00C2\u00A0at\t\r \u00C2\u00A0either\t\r \u00C2\u00A025\t\r \u00C2\u00A0or\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0920\t\r \u00C2\u00A0(21%)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0possessed\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0files\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A03,377\t\r \u00C2\u00A0(79%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0discovered\t\r \u00C2\u00A0on\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0but\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0themselves\t\r \u00C2\u00A0possess\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0files\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A08).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Table\t\r \u00C2\u00A08.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0categories\t\r \u00C2\u00A0 Risk\t\r \u00C2\u00A0Category\t\r \u00C2\u00A0 Seen\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0 Discovered\t\r \u00C2\u00A0on\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0 Proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0 (/100,000)\t\r \u00C2\u00A0 Proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0 (1/X)\t\r \u00C2\u00A0 50%\t\r \u00C2\u00A0 787\t\r \u00C2\u00A0 1465\t\r \u00C2\u00A0 2,252\t\r \u00C2\u00A0 49.2\t\r \u00C2\u00A0 1/2,047\t\r \u00C2\u00A0 25%\t\r \u00C2\u00A0 133\t\r \u00C2\u00A0 1912\t\r \u00C2\u00A0 2,045\t\r \u00C2\u00A0 44.7\t\r \u00C2\u00A0 1/2,254\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 920\t\r \u00C2\u00A0 3377\t\r \u00C2\u00A0 4,297\t\r \u00C2\u00A0 94.0\t\r \u00C2\u00A0 1/1,073\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0920\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0was\t\r \u00C2\u00A0known\t\r \u00C2\u00A0for\t\r \u00C2\u00A0859\t\r \u00C2\u00A0of\t\r \u00C2\u00A0them,\t\r \u00C2\u00A0762\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A097\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0315\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0in\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver;\t\r \u00C2\u00A045\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0447\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A052\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Using\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A01,075,589\t\r \u00C2\u00A0(23%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0total\t\r \u00C2\u00A0population)\t\r \u00C2\u00A0(British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Statistics,\t\r \u00C2\u00A02012),\t\r \u00C2\u00A0the\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A03,438\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0was\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0or\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0designation.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Twenty-\u00C2\u00AD\u00E2\u0080\u0090three\t\r \u00C2\u00A0percent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0and\t\r \u00C2\u00A077%,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0total,\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A01/536\t\r 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Rural=\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A02\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0away\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car;\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Urban=\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0920\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0seen\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0charts\t\r \u00C2\u00A0belonging\t\r \u00C2\u00A0to\t\r \u00C2\u00A0859\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0included\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0their\t\r \u00C2\u00A0provincial\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A03,438\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0distributed\t\r \u00C2\u00A0among\t\r \u00C2\u00A0health\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0according\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0general\t\r \u00C2\u00A0population\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0each\t\r \u00C2\u00A0region\t\r \u00C2\u00A0(the\t\r \u00C2\u00A0859\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0with\t\r \u00C2\u00A0known\t\r \u00C2\u00A0addresses\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0dispersed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0manner).\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A058\t\r \u00C2\u00A0(7%)\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region,\t\r \u00C2\u00A0236\t\r \u00C2\u00A0(27%),\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Coastal\t\r \u00C2\u00A0health;\t\r \u00C2\u00A0157\t\r \u00C2\u00A0(18%),\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Interior;\t\r \u00C2\u00A0128\t\r \u00C2\u00A0(15%)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0health\t\r \u00C2\u00A0and\t\r \u00C2\u00A0281\t\r \u00C2\u00A0(33%)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Fraser\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A019).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0roughly\t\r \u00C2\u00A01/1,074\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region;\t\r \u00C2\u00A01/1,029\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Coastal\t\r \u00C2\u00A0 Rural (23% population) Urban (77% population) Vancouver Victoria Prince George Risk% Empirical% %Total% Theore3cal% Total% 50%$ 315% 41%% 342$ 657$ 25%$ 45% 46%% 448$ 493$ 2,005$ Total$at$risk:$~1/536$ Risk% Empirical% %Total% Theore3cal% Total% 50%$ 447% 59%% 1,147$ 1,594$ 25%$ 52% 54%% 1,500$ 1,552$ 3,146$ Total$at$risk:$~1/1,123$ \t\r \u00C2\u00A0 52\t\r \u00C2\u00A0 health;\t\r \u00C2\u00A01/1,054\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Interior;\t\r \u00C2\u00A01/1,106\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0health\t\r \u00C2\u00A0and\t\r \u00C2\u00A01/1,094\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Fraser\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A019).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A019.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0each\t\r \u00C2\u00A0provincial\t\r \u00C2\u00A0health\t\r \u00C2\u00A0region\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Risk\t\r \u00C2\u00A0categories\t\r \u00C2\u00A0after\t\r \u00C2\u00A0accounting\t\r \u00C2\u00A0for\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results:\t\r \u00C2\u00A0When\t\r \u00C2\u00A0taking\t\r \u00C2\u00A0into\t\r \u00C2\u00A0account\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0along\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0CMMT\t\r \u00C2\u00A0research\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0negative/decreased\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Pre-\u00C2\u00AD\u00E2\u0080\u0090manifest\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0category\t\r \u00C2\u00A0are\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0analysis.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0344\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0make\t\r \u00C2\u00A0up\t\r \u00C2\u00A0the\t\r \u00C2\u00A0negative/decreased\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0category\t\r \u00C2\u00A0and\t\r \u00C2\u00A0326\t\r \u00C2\u00A0make\t\r \u00C2\u00A0up\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pre-\u00C2\u00AD\u00E2\u0080\u0090manifest\t\r \u00C2\u00A0category.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0326\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0with\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results,\t\r \u00C2\u00A036\t\r \u00C2\u00A0of\t\r \u00C2\u00A0them\t\r \u00C2\u00A0were\t\r \u00C2\u00A0discovered\t\r \u00C2\u00A0on\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0and\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0themselves\t\r \u00C2\u00A0possess\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0files.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0were\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0tested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0another\t\r \u00C2\u00A0province\t\r \u00C2\u00A0or\t\r \u00C2\u00A0country\t\r \u00C2\u00A0but\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0 Regional population at risk 58 (7%) 157 (18%) 281 (33%) 236 (27%) 128 (15%) 50%$ $ 25%$ $ $ Total$ $ Popula.on$$ at$risk$ (1/X)& Health$region$ $ Empirical$ Theore.cal$ Empirical$ Theore.cal$ 52& 101& 6& 133& 292& 1,074& Northern$ 210& 338& 26& 442& 1016& 1,029& Vancouver$Coastal$ 139& 251& 18& 328& 736& 1,054& Interior$ 109& 258& 19& 338& 724& 1,106& Vancouver$Island$ 253& 541& 28& 707& 1,529& 1,094& Fraser$ $$Total$ 2,252$ 2,045$ 4,297$ 1,073$ \t\r \u00C2\u00A0 53\t\r \u00C2\u00A0 BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Approximately\t\r \u00C2\u00A01/14,141\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0are\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0highly\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0to\t\r \u00C2\u00A0develop\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0future\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0confirmation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0mutation.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A03,627\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0have\t\r \u00C2\u00A0not\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test;\t\r \u00C2\u00A02,045\t\r \u00C2\u00A0remain\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A025%\t\r \u00C2\u00A0a-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A01,582\t\r \u00C2\u00A0with\t\r \u00C2\u00A0an\t\r \u00C2\u00A0a\t\r \u00C2\u00A0priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0of\t\r \u00C2\u00A050%\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A09).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Table\t\r \u00C2\u00A09.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Break\t\r \u00C2\u00A0down\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0categories\t\r \u00C2\u00A0after\t\r \u00C2\u00A0 accounting\t\r \u00C2\u00A0for\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Seen\t\r \u00C2\u00A0at\t\r \u00C2\u00A0 Clinic\t\r \u00C2\u00A0 Discovered\t\r \u00C2\u00A0on\t\r \u00C2\u00A0 pedigrees\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0 Proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0 (/100,000)\t\r \u00C2\u00A0 Proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0 (1/X)\t\r \u00C2\u00A0 Tested\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 0%\t\r \u00C2\u00A0(Direct\t\r \u00C2\u00A0mutation)\t\r \u00C2\u00A0 293\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 344\t\r \u00C2\u00A0 8.6\t\r \u00C2\u00A0 1/11,574\t\r \u00C2\u00A0 Negative\t\r \u00C2\u00A0(Linkage\t\r \u00C2\u00A0analysis)\t\r \u00C2\u00A0 51\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 100%\t\r \u00C2\u00A0 255\t\r \u00C2\u00A0 36\t\r \u00C2\u00A0 326\t\r \u00C2\u00A0 7.1\t\r \u00C2\u00A0 1/14,027\t\r \u00C2\u00A0Positive\t\r \u00C2\u00A0(Linkage\t\r \u00C2\u00A0analysis)\t\r \u00C2\u00A0 35\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 Untested\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 25%\t\r \u00C2\u00A0 95\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 2,045\t\r \u00C2\u00A0 43.9\t\r \u00C2\u00A0 1/2,278\t\r \u00C2\u00A0 50%\t\r \u00C2\u00A0 323\t\r \u00C2\u00A0 1259\t\r \u00C2\u00A0 1,582\t\r \u00C2\u00A0 38.3\t\r \u00C2\u00A0 1/2,611\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4297\t\r \u00C2\u00A0 94\t\r \u00C2\u00A0 1/1,073\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 54\t\r \u00C2\u00A0 Predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0A\t\r \u00C2\u00A0total\t\r \u00C2\u00A0of\t\r \u00C2\u00A0634\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbians\t\r \u00C2\u00A0have\t\r \u00C2\u00A0participated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0program\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A01987;\t\r \u00C2\u00A0290\t\r \u00C2\u00A0(46%)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0which\t\r \u00C2\u00A0revealed\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0and\t\r \u00C2\u00A0344\t\r \u00C2\u00A0(54%)\t\r \u00C2\u00A0of\t\r \u00C2\u00A0which\t\r \u00C2\u00A0revealed\t\r \u00C2\u00A0negative\t\r \u00C2\u00A0results.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0634\t\r \u00C2\u00A0total\t\r \u00C2\u00A0tests,\t\r \u00C2\u00A086\t\r \u00C2\u00A0(14%)\t\r \u00C2\u00A0were\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0via\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0and\t\r \u00C2\u00A0548\t\r \u00C2\u00A0(86%)\t\r \u00C2\u00A0via\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A020).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A020.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0between\t\r \u00C2\u00A01987\t\r \u00C2\u00A0and\t\r \u00C2\u00A0April\t\r \u00C2\u00A0 2012.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0bottom\t\r \u00C2\u00A0section\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0bar\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0top\t\r \u00C2\u00A0 portion\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 Uptake:\t\r \u00C2\u00A0The\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A012-\u00C2\u00AD\u00E2\u0080\u0090year\t\r \u00C2\u00A0period\t\r \u00C2\u00A0between\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02000\t\r \u00C2\u00A0and\t\r \u00C2\u00A0April\t\r \u00C2\u00A01,\t\r \u00C2\u00A02012\t\r \u00C2\u00A0using\t\r \u00C2\u00A0two\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0two\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Using\t\r \u00C2\u00A0the\t\r \u00C2\u00A0empirical\t\r \u00C2\u00A0data\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0to\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0at\t\r \u00C2\u00A021%\t\r \u00C2\u00A0and\t\r \u00C2\u00A013%,\t\r \u00C2\u00A0using\t\r \u00C2\u00A0the\t\r \u00C2\u00A0original\t\r \u00C2\u00A0formula\t\r \u00C2\u00A0and\t\r \u00C2\u00A0then\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0formula\t\r \u00C2\u00A0respectively\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003,\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0second\t\r \u00C2\u00A0method\t\r \u00C2\u00A0of\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0was\t\r \u00C2\u00A0to\t\r \u00C2\u00A0use\t\r \u00C2\u00A0theoretical\t\r \u00C2\u00A0data\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0multiply\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(13.9/100,000\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0an\t\r \u00C2\u00A0average\t\r \u00C2\u00A0of\t\r \u00C2\u00A0all\t\r \u00C2\u00A0three\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study)\t\r \u00C2\u00A0by\t\r \u00C2\u00A04.2.\t\r \u00C2\u00A0Using\t\r \u00C2\u00A0this\t\r \u00C2\u00A0method,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0was\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A018%\t\r \u00C2\u00A0and\t\r \u00C2\u00A011%\t\r \u00C2\u00A0with\t\r \u00C2\u00A0respect\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0original\t\r \u00C2\u00A0formula\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0formula\t\r \u00C2\u00A0revised\t\r \u00C2\u00A0by\t\r \u00C2\u00A0Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A010).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 35\t\r \u00C2\u00A0 51\t\r \u00C2\u00A0 86\t\r \u00C2\u00A0 255\t\r \u00C2\u00A0 293\t\r \u00C2\u00A0 548\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0100\t\r \u00C2\u00A0 200\t\r \u00C2\u00A0300\t\r \u00C2\u00A0 400\t\r \u00C2\u00A0500\t\r \u00C2\u00A0 600\t\r \u00C2\u00A0700\t\r \u00C2\u00A0 Positive\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0 Negative\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0 Total\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0Linkage\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0 Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 344 290 634 Numbe r\t\r \u00C2\u00A0 \t\r \u00C2\u00A0(of\t\r \u00C2\u00A0pre dictive \t\r \u00C2\u00A0tests\t\r \u00C2\u00A0p erform ed)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 55\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A010.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0(2000-\u00C2\u00AD\u00E2\u0080\u00902012)\t\r \u00C2\u00A0using\t\r \u00C2\u00A0two\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0 methods\t\r \u00C2\u00A0for\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0two\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0equations\t\r \u00C2\u00A0for\t\r \u00C2\u00A0 calculating\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 #\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0 at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0 the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0 Minus\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0<18\t\r \u00C2\u00A0 years\t\r \u00C2\u00A0 D\t\r \u00C2\u00A0 Uptake\t\r \u00C2\u00A0\t\r \u00C2\u00A0(0riginal)\t\r \u00C2\u00A0D/P\t\r \u00C2\u00A0 Uptake\t\r \u00C2\u00A0\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.)\t\r \u00C2\u00A0D/(P+P*t/18.8)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Empirical\t\r \u00C2\u00A0\t\r \u00C2\u00A0 (ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0BC)\t\r \u00C2\u00A0 2,252\t\r \u00C2\u00A0 1,351\t\r \u00C2\u00A0 280\t\r \u00C2\u00A0 21%\t\r \u00C2\u00A0 13%\t\r \u00C2\u00A0 Theoretical\t\r \u00C2\u00A0\t\r \u00C2\u00A0 (prevalence\t\r \u00C2\u00A0x4.2)\t\r \u00C2\u00A0 2,536\t\r \u00C2\u00A0 1,522\t\r \u00C2\u00A0 280\t\r \u00C2\u00A0 18%\t\r \u00C2\u00A0 11%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 D=\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0performed;\t\r \u00C2\u00A0P=\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0eligible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0(age\t\r \u00C2\u00A018\t\r \u00C2\u00A0or\t\r \u00C2\u00A0above);\t\r \u00C2\u00A0t=number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0years\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period;\t\r \u00C2\u00A018.8=\t\r \u00C2\u00A0average\t\r \u00C2\u00A0duration\t\r \u00C2\u00A0of\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0(HD\t\r \u00C2\u00A0symptoms)\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 56\t\r \u00C2\u00A0 Discussion\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0presented\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0of\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0ranges\t\r \u00C2\u00A0from\t\r \u00C2\u00A012.5-\u00C2\u00AD\u00E2\u0080\u009014.9/100,000\t\r \u00C2\u00A0(1/8,000-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A01/6,711)\t\r \u00C2\u00A0(mean=13.6/100,000\t\r \u00C2\u00A0(1/7,353),\t\r \u00C2\u00A095%CI:\t\r \u00C2\u00A011.9-\u00C2\u00AD\u00E2\u0080\u009016.0\t\r \u00C2\u00A0(1/8,403-\u00C2\u00AD\u00E2\u0080\u00901/6,250).\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0highest\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0world\t\r \u00C2\u00A0since\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0in\t\r \u00C2\u00A01993\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A021).\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0comparing\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0from\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0after\t\r \u00C2\u00A01993,\t\r \u00C2\u00A0only\t\r \u00C2\u00A0three\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0presented\t\r \u00C2\u00A0comparable\t\r \u00C2\u00A0findings\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0from\t\r \u00C2\u00A0BC:\t\r \u00C2\u00A0Malta,\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A021).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study\t\r \u00C2\u00A0is\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0both\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0(\u00CF\u00872=207.3,\t\r \u00C2\u00A0p<0.001,\t\r \u00C2\u00A0df=2)\t\r \u00C2\u00A0(Figure22).\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0also\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0those\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0\t\r \u00C2\u00A0(10.6/100,000,\t\r \u00C2\u00A095%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A09.1-\u00C2\u00AD\u00E2\u0080\u009012.1,\t\r \u00C2\u00A0\u00CF\u00872=9.1,\t\r \u00C2\u00A0p<0.01,\t\r \u00C2\u00A0df=1)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(12.4/100,000,\t\r \u00C2\u00A095%CI:\t\r \u00C2\u00A012.1-\u00C2\u00AD\u00E2\u0080\u009012.7,\t\r \u00C2\u00A0\u00CF\u00872=5.46,\t\r \u00C2\u00A0p<0.05,\t\r \u00C2\u00A0df=1)\t\r \u00C2\u00A0(Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010,\t\r \u00C2\u00A0Rawlins,\t\r \u00C2\u00A02010),\t\r \u00C2\u00A0but\t\r \u00C2\u00A0is\t\r \u00C2\u00A0not\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Malta\t\r \u00C2\u00A0(11.8,\t\r \u00C2\u00A095%CI:\t\r \u00C2\u00A08.3-\u00C2\u00AD\u00E2\u0080\u009015.3,\t\r \u00C2\u00A0\u00CF\u00872=0.95,\t\r \u00C2\u00A0p>0.05,\t\r \u00C2\u00A0df=1)\t\r \u00C2\u00A0(Gassivaro\t\r \u00C2\u00A0Gallo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01994).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0island\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Malta\t\r \u00C2\u00A0has\t\r \u00C2\u00A0a\t\r \u00C2\u00A0relatively\t\r \u00C2\u00A0small\t\r \u00C2\u00A0population\t\r \u00C2\u00A0size\t\r \u00C2\u00A0and\t\r \u00C2\u00A0has\t\r \u00C2\u00A0attributed\t\r \u00C2\u00A0its\t\r \u00C2\u00A0abnormally\t\r \u00C2\u00A0high\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0founder\t\r \u00C2\u00A0effect\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0Maritime\t\r \u00C2\u00A0traffic\t\r \u00C2\u00A0(Gassivaro\t\r \u00C2\u00A0Gallo,\t\r \u00C2\u00A01994).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Ascertainment\t\r \u00C2\u00A0efforts\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Irish\t\r \u00C2\u00A0study\t\r \u00C2\u00A0were\t\r \u00C2\u00A0limited\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0(Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0only\t\r \u00C2\u00A0a\t\r \u00C2\u00A0single\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0source,\t\r \u00C2\u00A0consisting\t\r \u00C2\u00A0of\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Association\t\r \u00C2\u00A0clientele\t\r \u00C2\u00A0count.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lack\t\r \u00C2\u00A0of\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0efforts\t\r \u00C2\u00A0and\t\r \u00C2\u00A0no\t\r \u00C2\u00A0conformation\t\r \u00C2\u00A0or\t\r \u00C2\u00A0follow-\u00C2\u00AD\u00E2\u0080\u0090up\t\r \u00C2\u00A0of\t\r \u00C2\u00A0cases,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0requires\t\r \u00C2\u00A0repetition\t\r \u00C2\u00A0with\t\r \u00C2\u00A0improved\t\r \u00C2\u00A0methodology\t\r \u00C2\u00A0to\t\r \u00C2\u00A0achieve\t\r \u00C2\u00A0more\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0results\t\r \u00C2\u00A0(Rawlins\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 57\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A021.\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0region\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time\t\r \u00C2\u00A0including\t\r \u00C2\u00A0 updated\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 Prevale nce\t\r \u00C2\u00A0(/1 00,000 )\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A04.8\t\r \u00C2\u00A02.7\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 1.3\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 4.5\t\r 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San-\u00C2\u00AD\u00E2\u0080\u0090in) \t\r \u00C2\u00A0 Austra lia\t\r \u00C2\u00A0 N.\t\r \u00C2\u00A0Irela nd\t\r \u00C2\u00A0 Croatia \t\r \u00C2\u00A0 Sloven ia\t\r \u00C2\u00A0 Taiwan \t\r \u00C2\u00A0 Greece \t\r \u00C2\u00A0 Mexico \t\r \u00C2\u00A0 Venezu ela\t\r \u00C2\u00A0 UK\t\r \u00C2\u00A0 UK\t\r \u00C2\u00A0 BC\t\r \u00C2\u00A01930\t\r \u00C2\u00A0 2012\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available\t\r \u00C2\u00A0\t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A022.\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0Error\t\r \u00C2\u00A0bars\t\r \u00C2\u00A0 represent\t\r \u00C2\u00A095%\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0intervals\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 13.6\t\r \u00C2\u00A0 0\t\r \u00C2\u00A02\t\r \u00C2\u00A0 4\t\r \u00C2\u00A06\t\r \u00C2\u00A0 8\t\r \u00C2\u00A010\t\r \u00C2\u00A0 12\t\r \u00C2\u00A014\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 1975\t\r \u00C2\u00A0 2012\t\r \u00C2\u00A0Quebec\t\r \u00C2\u00A0 Canadian\t\r \u00C2\u00A0Prairies\t\r \u00C2\u00A0 BC\t\r \u00C2\u00A0 Prevale nce\t\r \u00C2\u00A0(/1 00,000 )\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test \t\r \u00C2\u00A0 58\t\r \u00C2\u00A0 An\t\r \u00C2\u00A0important\t\r \u00C2\u00A0question\t\r \u00C2\u00A0to\t\r \u00C2\u00A0ponder\t\r 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\u00C2\u00A0increased\t\r \u00C2\u00A0by\t\r \u00C2\u00A016\t\r \u00C2\u00A0years\t\r \u00C2\u00A0and\t\r \u00C2\u00A0by\t\r \u00C2\u00A010\t\r \u00C2\u00A0years\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0alone\t\r \u00C2\u00A0since\t\r \u00C2\u00A0the\t\r \u00C2\u00A01960\u00E2\u0080\u0099s\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A023).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Taken\t\r \u00C2\u00A0together,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0aging\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0tendency\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0to\t\r \u00C2\u00A0manifest\t\r \u00C2\u00A0in\t\r \u00C2\u00A0mid-\u00C2\u00AD\u00E2\u0080\u0090life,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0experiencing\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0at\t\r \u00C2\u00A0any\t\r \u00C2\u00A0given\t\r \u00C2\u00A0time\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0increased.\t\r \u00C2\u00A0\t\r \u00C2\u00A0With\t\r \u00C2\u00A0an\t\r \u00C2\u00A0increasing\t\r \u00C2\u00A0life\t\r \u00C2\u00A0expectancy\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0living\t\r \u00C2\u00A0longer\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0an\t\r \u00C2\u00A0elevated\t\r \u00C2\u00A0possibility\t\r \u00C2\u00A0for\t\r \u00C2\u00A0late\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0to\t\r \u00C2\u00A0manifest\t\r \u00C2\u00A0before\t\r \u00C2\u00A0death.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Both\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0may\t\r \u00C2\u00A0contribute\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0true\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0New\t\r \u00C2\u00A0mutations\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0to\t\r \u00C2\u00A0arise\t\r \u00C2\u00A0at\t\r \u00C2\u00A0a\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A010%\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0(Falush\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000).\t\r \u00C2\u00A0\t\r \u00C2\u00A0New\t\r \u00C2\u00A0mutations\t\r \u00C2\u00A0introduced\t\r \u00C2\u00A0into\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0have\t\r \u00C2\u00A0the\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0to\t\r \u00C2\u00A0further\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Factors\t\r \u00C2\u00A0that\t\r \u00C2\u00A0may\t\r \u00C2\u00A0contribute\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0perceived\t\r \u00C2\u00A0rather\t\r \u00C2\u00A0than\t\r \u00C2\u00A0true\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0are\t\r \u00C2\u00A0the\t\r \u00C2\u00A0improved\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0for,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0rigor\t\r \u00C2\u00A0in,\t\r \u00C2\u00A0obtaining\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0past.\t\r \u00C2\u00A0\t\r \u00C2\u00A0There\t\r \u00C2\u00A0is\t\r \u00C2\u00A0ample\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0for\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0single\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0is\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0performing\t\r \u00C2\u00A0all\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0entire\t\r \u00C2\u00A0province\t\r \u00C2\u00A0and\t\r \u00C2\u00A0specialized\t\r \u00C2\u00A0professionals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0only\t\r \u00C2\u00A0two\t\r \u00C2\u00A0facilities\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0HD-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0care\t\r \u00C2\u00A0to\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0families\t\r \u00C2\u00A0from\t\r \u00C2\u00A0all\t\r \u00C2\u00A0around\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0this\t\r \u00C2\u00A0disease.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0was\t\r \u00C2\u00A0shown\t\r \u00C2\u00A0that\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0had\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0a\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0total\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0(predictive\t\r \u00C2\u00A0and\t\r \u00C2\u00A0diagnostic)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD,\t\r \u00C2\u00A0proportional\t\r \u00C2\u00A0to\t\r \u00C2\u00A0its\t\r \u00C2\u00A0population\t\r \u00C2\u00A0size,\t\r \u00C2\u00A0than\t\r \u00C2\u00A0any\t\r \u00C2\u00A0other\t\r \u00C2\u00A0province\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada.\t\r \u00C2\u00A0\t\r \u00C2\u00A0However,\t\r \u00C2\u00A0Quebec\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Alberta\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Having\t\r \u00C2\u00A0been\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0province\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0population\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0awareness\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 59\t\r \u00C2\u00A0 test\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0other\t\r \u00C2\u00A0provinces,\t\r \u00C2\u00A0further\t\r \u00C2\u00A0emphasizing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0high\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0for\t\r \u00C2\u00A0full\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Furthermore,\t\r \u00C2\u00A0when\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0assessments,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0variation\t\r \u00C2\u00A0in\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0were\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Multiple\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0communication\t\r \u00C2\u00A0and\t\r \u00C2\u00A0nine\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0were\t\r \u00C2\u00A0utilized\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0whereas\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0employed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0all\t\r \u00C2\u00A0other\t\r \u00C2\u00A0worldwide\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0after\t\r \u00C2\u00A01993\t\r \u00C2\u00A0ranged\t\r \u00C2\u00A0only\t\r \u00C2\u00A0from\t\r \u00C2\u00A0one\t\r \u00C2\u00A0to\t\r \u00C2\u00A0three\t\r \u00C2\u00A0\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Ten\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A014\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Table\t\r \u00C2\u00A011\t\r \u00C2\u00A0obtained\t\r \u00C2\u00A0results\t\r \u00C2\u00A0from\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0alone\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0exception\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0which\t\r \u00C2\u00A0used\t\r \u00C2\u00A0both\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0records.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0either\t\r \u00C2\u00A0used\t\r \u00C2\u00A0hospital\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0records\t\r \u00C2\u00A0alone\t\r \u00C2\u00A0or\t\r \u00C2\u00A0other\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Only\t\r \u00C2\u00A0two\t\r \u00C2\u00A0and\t\r \u00C2\u00A0five\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0were\t\r \u00C2\u00A0employed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0(Barbeau\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01964,\t\r \u00C2\u00A0Shokeir\t\r \u00C2\u00A01975).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Although\t\r \u00C2\u00A0the\t\r \u00C2\u00A0aging\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0new\t\r \u00C2\u00A0mutations\t\r \u00C2\u00A0have\t\r \u00C2\u00A0the\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0to\t\r \u00C2\u00A0contribute\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0elevated\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0these\t\r \u00C2\u00A0factors\t\r \u00C2\u00A0are\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0not\t\r \u00C2\u00A0weighted\t\r \u00C2\u00A0enough\t\r \u00C2\u00A0to\t\r \u00C2\u00A0produce\t\r \u00C2\u00A0the\t\r \u00C2\u00A0magnitude\t\r \u00C2\u00A0of\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0here.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0is\t\r \u00C2\u00A0primarily\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0improved\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0and\t\r \u00C2\u00A0represents\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0close\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0closest\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0issue\t\r \u00C2\u00A0worth\t\r \u00C2\u00A0questioning\t\r \u00C2\u00A0however,\t\r \u00C2\u00A0is\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0reason\t\r \u00C2\u00A0to\t\r \u00C2\u00A0believe\t\r \u00C2\u00A0that\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0represents\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence;\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0the\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0research\t\r \u00C2\u00A0and\t\r \u00C2\u00A0care\t\r \u00C2\u00A0facilities\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province\t\r \u00C2\u00A0act\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0motivating\t\r \u00C2\u00A0force,\t\r \u00C2\u00A0pulling\t\r \u00C2\u00A0more\t\r \u00C2\u00A0families\t\r \u00C2\u00A0to\t\r \u00C2\u00A0migrate\t\r \u00C2\u00A0to\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0From\t\r \u00C2\u00A0the\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0province-\u00C2\u00AD\u00E2\u0080\u0090wide\t\r \u00C2\u00A0review\t\r \u00C2\u00A0that\t\r \u00C2\u00A0took\t\r \u00C2\u00A0place\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0full\t\r \u00C2\u00A0history\t\r \u00C2\u00A0was\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0509\t\r \u00C2\u00A0chart-\u00C2\u00AD\u00E2\u0080\u0090reviewed\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0None\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0histories\t\r \u00C2\u00A0reviewed\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0migration\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0families\t\r \u00C2\u00A0to\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0purposes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0acquiring\t\r \u00C2\u00A0enhanced\t\r \u00C2\u00A0care\t\r \u00C2\u00A0or\t\r \u00C2\u00A0of\t\r \u00C2\u00A0seeking\t\r \u00C2\u00A0opportunities\t\r \u00C2\u00A0to\t\r \u00C2\u00A0participate\t\r \u00C2\u00A0in\t\r \u00C2\u00A0research.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0can\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0be\t\r \u00C2\u00A0assumed\t\r \u00C2\u00A0at\t\r \u00C2\u00A0this\t\r \u00C2\u00A0time\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0presented\t\r \u00C2\u00A0here\t\r \u00C2\u00A0is\t\r \u00C2\u00A0not\t\r \u00C2\u00A0overestimated\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0abnormally\t\r \u00C2\u00A0large\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0presence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further\t\r \u00C2\u00A0work\t\r \u00C2\u00A0involving\t\r \u00C2\u00A0a\t\r \u00C2\u00A0province-\u00C2\u00AD\u00E2\u0080\u0090wide\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0community\t\r \u00C2\u00A0including\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0related\t\r \u00C2\u00A0to\t\r \u00C2\u00A0family\t\r \u00C2\u00A0migration\t\r \u00C2\u00A0patterns\t\r \u00C2\u00A0would\t\r \u00C2\u00A0be\t\r \u00C2\u00A0required\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0gain\t\r \u00C2\u00A0a\t\r \u00C2\u00A0clearer\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0notion.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 60\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A023.\t\r \u00C2\u00A0Life\t\r \u00C2\u00A0expectancy\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0and\t\r \u00C2\u00A0worldwide\t\r \u00C2\u00A0(1960-\u00C2\u00AD\u00E2\u0080\u00902011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 52.65\t\r \u00C2\u00A0 62.95\t\r \u00C2\u00A0 67.23\t\r \u00C2\u00A0 69.41\t\r \u00C2\u00A0 69.41\t\r \u00C2\u00A0 71.4\t\r \u00C2\u00A0 73.5\t\r \u00C2\u00A0 76.7\t\r \u00C2\u00A0 78.9\t\r \u00C2\u00A0 81.4\t\r \u00C2\u00A0 0\t\r \u00C2\u00A010\t\r \u00C2\u00A0 20\t\r \u00C2\u00A030\t\r \u00C2\u00A0 40\t\r \u00C2\u00A050\t\r \u00C2\u00A0 60\t\r \u00C2\u00A070\t\r \u00C2\u00A0 80\t\r \u00C2\u00A090\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 2011\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Life\t\r \u00C2\u00A0expectancy\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada Life\t\r \u00C2\u00A0expectancy\t\r \u00C2\u00A0worldwide Age\t\r \u00C2\u00A0(ye ars)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 61\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A011.\t\r \u00C2\u00A0Comparison\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0employed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0after\t\r \u00C2\u00A0 the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0 Study\t\r \u00C2\u00A0region\t\r \u00C2\u00A0 and\t\r \u00C2\u00A0year\t\r \u00C2\u00A0 Prevalence\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0 Genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0records\t\r \u00C2\u00A0 Hosp.\t\r \u00C2\u00A0records\t\r \u00C2\u00A0 GP\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Neurol.\t\r \u00C2\u00A0records\t\r \u00C2\u00A0 HD\t\r \u00C2\u00A0services\t\r \u00C2\u00A0 ICD\t\r \u00C2\u00A0codes\t\r \u00C2\u00A0 Nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0 HD\t\r \u00C2\u00A0families\t\r \u00C2\u00A0 Pedigree\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A02012\t\r \u00C2\u00A0 12.5-\u00C2\u00AD\u00E2\u0080\u009014.9\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0The\t\r \u00C2\u00A0UK\t\r \u00C2\u00A02010\t\r \u00C2\u00A0 12.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0The\t\r \u00C2\u00A0UK\t\r \u00C2\u00A02008\t\r \u00C2\u00A0 6.2\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mexico\t\r \u00C2\u00A02008\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Taiwan\t\r \u00C2\u00A02007\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A02007\t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Slovenia\t\r \u00C2\u00A02006\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Greece\t\r \u00C2\u00A02002\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Croatia\t\r \u00C2\u00A02002\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0N.\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0 10.6\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Japan\t\r \u00C2\u00A01997\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Australia\t\r \u00C2\u00A01996\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0New\t\r \u00C2\u00A0Zealand\t\r \u00C2\u00A01996\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Germany\t\r \u00C2\u00A01995\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Switzerland\t\r \u00C2\u00A01995\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Malta\t\r \u00C2\u00A01994\t\r \u00C2\u00A0 11.8\t\r \u00C2\u00A0 \u00E2\u0088\u009A \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Ethnic-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0prevalence:\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0interesting\t\r \u00C2\u00A0to\t\r \u00C2\u00A0compare\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0minorities\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0groups\t\r \u00C2\u00A0as\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0countries\t\r \u00C2\u00A0of\t\r \u00C2\u00A0origin.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ethnic-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0from\t\r \u00C2\u00A0each\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0group\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0used\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0denominator\t\r \u00C2\u00A0for\t\r \u00C2\u00A0these\t\r \u00C2\u00A0calculations.\t\r \u00C2\u00A0Roughly\t\r \u00C2\u00A020%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\u00E2\u0080\u0099s\t\r \u00C2\u00A0population\t\r \u00C2\u00A0is\t\r \u00C2\u00A0made\t\r \u00C2\u00A0up\t\r \u00C2\u00A0of\t\r \u00C2\u00A0nationalities\t\r \u00C2\u00A0fitting\t\r \u00C2\u00A0into\t\r \u00C2\u00A0the\t\r \u00C2\u00A0East-\u00C2\u00AD\u00E2\u0080\u0090Asian\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0category\t\r \u00C2\u00A0(EHDN\t\r \u00C2\u00A02010,\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 62\t\r \u00C2\u00A0 Canada\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Asian\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A00.64/100,000\t\r \u00C2\u00A0(1/156,250).\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0expected,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0much\t\r \u00C2\u00A0smaller\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Caucasians\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0similar\t\r \u00C2\u00A0to,\t\r \u00C2\u00A0although\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0than,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region\t\r \u00C2\u00A0(0.42/100,000\t\r \u00C2\u00A0(1/238,095),\t\r \u00C2\u00A0Appendix\t\r \u00C2\u00A01b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0West-\u00C2\u00AD\u00E2\u0080\u0090Asian\t\r \u00C2\u00A0category\t\r \u00C2\u00A0showed\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A03.0/100,000\t\r \u00C2\u00A0(1/33,000);\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region,\t\r \u00C2\u00A0of\t\r \u00C2\u00A01.7/100,000\t\r \u00C2\u00A0(1/58,824)\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Latin-\u00C2\u00AD\u00E2\u0080\u0090American\t\r \u00C2\u00A0population\t\r \u00C2\u00A0showed\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A04.1/100,000\t\r \u00C2\u00A0(1/24,390),\t\r \u00C2\u00A0also\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0that\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region;\t\r \u00C2\u00A02.3/100,000\t\r \u00C2\u00A0(1/43,478)\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0North\t\r \u00C2\u00A0African\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A04.2/100,000\t\r \u00C2\u00A0(1/23,810),\t\r \u00C2\u00A0also\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0average\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r \u00C2\u00A0region\t\r \u00C2\u00A0of\t\r \u00C2\u00A03.6/100,000\t\r \u00C2\u00A0(1/28,000)\t\r \u00C2\u00A0(Appendix\t\r \u00C2\u00A01b).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0only\t\r \u00C2\u00A0minority\t\r \u00C2\u00A0without\t\r \u00C2\u00A0a\t\r \u00C2\u00A0previous\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0comparison\t\r \u00C2\u00A0was\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Canadian-\u00C2\u00AD\u00E2\u0080\u0090Aboriginal\t\r \u00C2\u00A0group\t\r \u00C2\u00A0in\t\r \u00C2\u00A0which\t\r \u00C2\u00A0a\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A00.60/100,000\t\r \u00C2\u00A0(1/167,000)\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0ethnic\t\r \u00C2\u00A0groups\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0those\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0from\t\r \u00C2\u00A0their\t\r \u00C2\u00A0region\t\r \u00C2\u00A0of\t\r \u00C2\u00A0origin\t\r \u00C2\u00A0may\t\r \u00C2\u00A0reflect\t\r \u00C2\u00A0cultural\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0adherence\t\r \u00C2\u00A0to\t\r \u00C2\u00A0health\t\r \u00C2\u00A0care.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Perhaps\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0less\t\r \u00C2\u00A0common\t\r \u00C2\u00A0to\t\r \u00C2\u00A0make\t\r \u00C2\u00A0public\t\r \u00C2\u00A0a\t\r \u00C2\u00A0familial\t\r \u00C2\u00A0degenerative\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0other\t\r \u00C2\u00A0cultures\t\r \u00C2\u00A0than\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0here\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further\t\r \u00C2\u00A0work\t\r \u00C2\u00A0is\t\r \u00C2\u00A0required\t\r \u00C2\u00A0to\t\r \u00C2\u00A0gain\t\r \u00C2\u00A0a\t\r \u00C2\u00A0better\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0differences.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Regional\t\r \u00C2\u00A0prevalence:\t\r \u00C2\u00A0when\t\r \u00C2\u00A0observing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A03\t\r \u00C2\u00A0times\t\r \u00C2\u00A0higher.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0may\t\r \u00C2\u00A0either\t\r \u00C2\u00A0be\t\r \u00C2\u00A0a\t\r \u00C2\u00A0reflection\t\r \u00C2\u00A0of\t\r \u00C2\u00A0under-\u00C2\u00AD\u00E2\u0080\u0090ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0or\t\r \u00C2\u00A0it\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0an\t\r \u00C2\u00A0indication\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0does\t\r \u00C2\u00A0in\t\r \u00C2\u00A0fact\t\r \u00C2\u00A0have\t\r \u00C2\u00A0a\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0maintain\t\r \u00C2\u00A0legal\t\r \u00C2\u00A0and\t\r \u00C2\u00A0ethical\t\r \u00C2\u00A0obligations,\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0information\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0outside\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0two\t\r \u00C2\u00A0clinics\t\r \u00C2\u00A0from\t\r \u00C2\u00A0which\t\r \u00C2\u00A0special\t\r \u00C2\u00A0consent\t\r \u00C2\u00A0was\t\r \u00C2\u00A0granted\t\r \u00C2\u00A0(UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH).\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0records\t\r \u00C2\u00A0were\t\r \u00C2\u00A0only\t\r \u00C2\u00A0crosschecked\t\r \u00C2\u00A0and\t\r \u00C2\u00A0distinguished\t\r \u00C2\u00A0via\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Contacting\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0from\t\r \u00C2\u00A0large\t\r \u00C2\u00A0metropolitan\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0(i.e.\t\r \u00C2\u00A0Vancouver)\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0feasible\t\r \u00C2\u00A0as\t\r \u00C2\u00A0there\t\r \u00C2\u00A0was\t\r \u00C2\u00A0no\t\r \u00C2\u00A0way\t\r \u00C2\u00A0to\t\r \u00C2\u00A0distinguish\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0one\t\r \u00C2\u00A0another.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0example,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0GP,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0psychiatrist\t\r \u00C2\u00A0may\t\r \u00C2\u00A0report\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0and\t\r \u00C2\u00A0there\t\r \u00C2\u00A0would\t\r \u00C2\u00A0be\t\r \u00C2\u00A0no\t\r \u00C2\u00A0way\t\r \u00C2\u00A0to\t\r \u00C2\u00A0avoid\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0and\t\r \u00C2\u00A0double\t\r \u00C2\u00A0counting.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0this\t\r \u00C2\u00A0shortfall,\t\r \u00C2\u00A0only\t\r \u00C2\u00A0neurologists\t\r \u00C2\u00A0from\t\r \u00C2\u00A0these\t\r \u00C2\u00A0large\t\r \u00C2\u00A0metropolitan\t\r \u00C2\u00A0centers\t\r \u00C2\u00A0were\t\r \u00C2\u00A0contacted;\t\r \u00C2\u00A0GPs\t\r \u00C2\u00A0and\t\r \u00C2\u00A0other\t\r \u00C2\u00A0specialists\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 63\t\r \u00C2\u00A0 may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0led\t\r \u00C2\u00A0to\t\r \u00C2\u00A0under\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is,\t\r \u00C2\u00A0however,\t\r \u00C2\u00A0expected\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0would\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Thus,\t\r \u00C2\u00A0if\t\r \u00C2\u00A0under-\u00C2\u00AD\u00E2\u0080\u0090ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0factor,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0unlikely\t\r \u00C2\u00A0that\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0extreme\t\r \u00C2\u00A0enough\t\r \u00C2\u00A0to\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0the\t\r \u00C2\u00A0abrupt\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0between\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0here.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0the\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0does\t\r \u00C2\u00A0in\t\r \u00C2\u00A0fact\t\r \u00C2\u00A0reflect\t\r \u00C2\u00A0a\t\r \u00C2\u00A0true\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0between\t\r \u00C2\u00A0the\t\r \u00C2\u00A0two\t\r \u00C2\u00A0regions,\t\r \u00C2\u00A0there\t\r \u00C2\u00A0are\t\r \u00C2\u00A0a\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0contributory\t\r \u00C2\u00A0factors.\t\r \u00C2\u00A0\t\r \u00C2\u00A0First,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0that\t\r \u00C2\u00A0families\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0may\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0able\t\r \u00C2\u00A0to\t\r \u00C2\u00A0afford\t\r \u00C2\u00A0living\t\r \u00C2\u00A0within\t\r \u00C2\u00A02\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0families\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0too\t\r \u00C2\u00A0large\t\r \u00C2\u00A0a\t\r \u00C2\u00A0financial\t\r \u00C2\u00A0burden\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0caring\t\r \u00C2\u00A0for\t\r \u00C2\u00A0those\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0and\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0family,\t\r \u00C2\u00A0that\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0might\t\r \u00C2\u00A0be\t\r \u00C2\u00A0the\t\r \u00C2\u00A0only\t\r \u00C2\u00A0option\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0financially\t\r \u00C2\u00A0feasible.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Another\t\r \u00C2\u00A0factor\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lifestyle\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0with\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0living.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition,\t\r \u00C2\u00A0more\t\r \u00C2\u00A0personal\t\r \u00C2\u00A0space\t\r \u00C2\u00A0and\t\r \u00C2\u00A0less\t\r \u00C2\u00A0crowd-\u00C2\u00AD\u00E2\u0080\u0090density\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0more\t\r \u00C2\u00A0suitable\t\r \u00C2\u00A0for\t\r \u00C2\u00A0families\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0urban\t\r \u00C2\u00A0living.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0may\t\r \u00C2\u00A0also\t\r \u00C2\u00A0feel\t\r \u00C2\u00A0more\t\r \u00C2\u00A0comfortable\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0rural\t\r \u00C2\u00A0environment\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0social\t\r \u00C2\u00A0stigma\t\r \u00C2\u00A0associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0more\t\r \u00C2\u00A0emphasized\t\r \u00C2\u00A0in\t\r \u00C2\u00A0urban\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0with\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0crowd\t\r \u00C2\u00A0density\t\r \u00C2\u00A0than\t\r \u00C2\u00A0in\t\r \u00C2\u00A0rural\t\r \u00C2\u00A0regions.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0explore\t\r \u00C2\u00A0these\t\r 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\u00C2\u00A0Island\u00E2\u0080\u0099s\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0reside\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Since\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0constitutes\t\r \u00C2\u00A0only\t\r \u00C2\u00A0~12%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island\u00E2\u0080\u0099s\t\r \u00C2\u00A0population,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0led\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0abrupt\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence;\t\r \u00C2\u00A082.5/100,000\t\r \u00C2\u00A0(1/1,212)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0and\t\r \u00C2\u00A09.1/100,000\t\r \u00C2\u00A0(1/11,000)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0Island.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0information\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0the\t\r \u00C2\u00A0preference\t\r \u00C2\u00A0for\t\r \u00C2\u00A0rural\t\r \u00C2\u00A0living\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0not\t\r \u00C2\u00A0a\t\r \u00C2\u00A0factor\t\r \u00C2\u00A0contributing\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0extreme\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0in\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0for\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0whole.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0cost\t\r \u00C2\u00A0of\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0is\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0than\t\r \u00C2\u00A0the\t\r \u00C2\u00A0cost\t\r \u00C2\u00A0of\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Perhaps\t\r \u00C2\u00A0the\t\r \u00C2\u00A0high\t\r \u00C2\u00A0cost\t\r \u00C2\u00A0of\t\r \u00C2\u00A0living\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0dominant\t\r \u00C2\u00A0factor\t\r \u00C2\u00A0leading\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0relatively\t\r \u00C2\u00A0low\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0area.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0addition,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0abrupt\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0related\t\r \u00C2\u00A0in\t\r \u00C2\u00A0part\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0definitions\t\r \u00C2\u00A0of\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Cities\t\r \u00C2\u00A0designated\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0category\t\r \u00C2\u00A0were\t\r \u00C2\u00A0all\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0away\t\r \u00C2\u00A0from\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 64\t\r \u00C2\u00A0 Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car,\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0including\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0with\t\r \u00C2\u00A0larger\t\r \u00C2\u00A0populations,\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0Victoria,\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0generally\t\r \u00C2\u00A0defined\t\r \u00C2\u00A0as\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Findings\t\r \u00C2\u00A0from\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0suggest\t\r \u00C2\u00A0the\t\r \u00C2\u00A0vast\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0living\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0may\t\r \u00C2\u00A0suggest\t\r \u00C2\u00A0that\t\r \u00C2\u00A0participation\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0is\t\r \u00C2\u00A0only\t\r \u00C2\u00A0accessible\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0small\t\r \u00C2\u00A0fraction\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\u00E2\u0080\u0099s\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0However,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0also\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0that\t\r \u00C2\u00A0this\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0an\t\r \u00C2\u00A0under-\u00C2\u00AD\u00E2\u0080\u0090ascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0expected\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0proportions\t\r \u00C2\u00A0should\t\r \u00C2\u00A0be\t\r \u00C2\u00A0equal.\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0the\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0was\t\r \u00C2\u00A0repeated\t\r \u00C2\u00A0by\t\r \u00C2\u00A0placing\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0continued\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0nearly\t\r \u00C2\u00A03-\u00C2\u00AD\u00E2\u0080\u0090times\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0although\t\r \u00C2\u00A0the\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0was\t\r \u00C2\u00A0less\t\r \u00C2\u00A0extreme.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Despite\t\r \u00C2\u00A0the\t\r \u00C2\u00A0extensive\t\r \u00C2\u00A0efforts\t\r \u00C2\u00A0put\t\r \u00C2\u00A0forth\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0to\t\r \u00C2\u00A0thoroughly\t\r \u00C2\u00A0search\t\r \u00C2\u00A0for\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r 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\u00C2\u00A0of\t\r \u00C2\u00A0residence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0that\t\r \u00C2\u00A0some\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0moved\t\r \u00C2\u00A0to\t\r \u00C2\u00A0a\t\r \u00C2\u00A0different\t\r \u00C2\u00A0city\t\r \u00C2\u00A0causing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0to\t\r \u00C2\u00A0lack\t\r \u00C2\u00A0accuracy.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0homes:\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0some\t\r \u00C2\u00A0instances,\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0staff\t\r \u00C2\u00A0was\t\r \u00C2\u00A0unaware\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0information\t\r \u00C2\u00A0required\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0overlap.\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0under\t\r \u00C2\u00A0these\t\r \u00C2\u00A0circumstances\t\r \u00C2\u00A0were\t\r \u00C2\u00A0assigned\t\r \u00C2\u00A0an\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0of\t\r \u00C2\u00A02.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0possible\t\r \u00C2\u00A0that,\t\r \u00C2\u00A0although\t\r \u00C2\u00A0multiple\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0were\t\r \u00C2\u00A0utilized\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0we\t\r \u00C2\u00A0have\t\r \u00C2\u00A0not\t\r \u00C2\u00A0achieved\t\r \u00C2\u00A0full\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0full\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0were\t\r \u00C2\u00A0achieved,\t\r \u00C2\u00A0no\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0between\t\r 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\u00C2\u00A0\t\r \u00C2\u00A0Achieving\t\r \u00C2\u00A0full\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0would\t\r \u00C2\u00A0require\t\r \u00C2\u00A0the\t\r \u00C2\u00A0surveying\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090surveying\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0(66%)\t\r \u00C2\u00A0who\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0would\t\r \u00C2\u00A0also\t\r \u00C2\u00A0require\t\r \u00C2\u00A0ethical\t\r \u00C2\u00A0approval\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0collection\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0information\t\r \u00C2\u00A0from\t\r \u00C2\u00A0all\t\r 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\u00C2\u00A0questions\t\r \u00C2\u00A0about\t\r \u00C2\u00A0their\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0avoid\t\r \u00C2\u00A0overlap.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Incidence\t\r \u00C2\u00A0An\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A07.2\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0was\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study;\t\r \u00C2\u00A0this\t\r \u00C2\u00A0appears\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0a\t\r \u00C2\u00A0slight\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0from\t\r \u00C2\u00A0that\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0study\t\r \u00C2\u00A0(Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001).\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important,\t\r \u00C2\u00A0however,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0note\t\r \u00C2\u00A0that\t\r \u00C2\u00A0Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0counted\t\r \u00C2\u00A0incident\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0only\t\r \u00C2\u00A0if\t\r \u00C2\u00A0they\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Figure\t\r \u00C2\u00A024a\t\r \u00C2\u00A0shows\t\r \u00C2\u00A0the\t\r \u00C2\u00A0yearly\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A01987\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0used\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study;\t\r \u00C2\u00A0incident\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0include\t\r \u00C2\u00A0those\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0via\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0as\t\r \u00C2\u00A0well\t\r \u00C2\u00A0as\t\r \u00C2\u00A0those\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test.\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0comparing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0using\t\r \u00C2\u00A0this\t\r \u00C2\u00A0method,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0becomes\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0increased\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0period\t\r \u00C2\u00A0(1987-\u00C2\u00AD\u00E2\u0080\u00901992)\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0second\t\r \u00C2\u00A0period\t\r 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\u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A02011\t\r \u00C2\u00A0seems\t\r \u00C2\u00A0very\t\r \u00C2\u00A0low\t\r \u00C2\u00A0and\t\r \u00C2\u00A0when\t\r \u00C2\u00A0removed,\t\r \u00C2\u00A0recovers\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Incidence\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0underestimated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0year\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0timing\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0in\t\r \u00C2\u00A0having\t\r \u00C2\u00A0test\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0sent\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0lab\t\r 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\u00C2\u00A0this\t\r \u00C2\u00A0time,\t\r \u00C2\u00A0leading\t\r \u00C2\u00A0to\t\r \u00C2\u00A0an\t\r \u00C2\u00A0underestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence.\t\r \u00C2\u00A0Similar\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0also\t\r \u00C2\u00A0appears\t\r \u00C2\u00A0to\t\r \u00C2\u00A0fluctuate\t\r \u00C2\u00A0across\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0temporal\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A025).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0two\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0USA,\t\r \u00C2\u00A0were\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0before\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0became\t\r \u00C2\u00A0available\t\r \u00C2\u00A0and\t\r \u00C2\u00A0both\t\r \u00C2\u00A0report\t\r \u00C2\u00A0a\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0than\t\r \u00C2\u00A0those\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0after.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 68\t\r \u00C2\u00A0 This\t\r \u00C2\u00A0may\t\r \u00C2\u00A0reflect\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ability\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0to\t\r \u00C2\u00A0diagnose\t\r \u00C2\u00A0atypical\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0that\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0missed\t\r \u00C2\u00A0before.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A024a.\t\r \u00C2\u00A0Estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A01987\t\r \u00C2\u00A0using\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0 diagnostic\t\r \u00C2\u00A0criteria.\t\r \u00C2\u00A0Error\t\r \u00C2\u00A0bars\t\r \u00C2\u00A0represent\t\r \u00C2\u00A095%\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0intervals\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3.9\t\r \u00C2\u00A0 7.4\t\r \u00C2\u00A06.9\t\r \u00C2\u00A04.9\t\r \u00C2\u00A03.9\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A05.9\t\r \u00C2\u00A0 9.0\t\r \u00C2\u00A07.9\t\r \u00C2\u00A09.3\t\r \u00C2\u00A06.8\t\r \u00C2\u00A0 8.0\t\r \u00C2\u00A07.5\t\r \u00C2\u00A06.9\t\r \u00C2\u00A06.9\t\r \u00C2\u00A08.8\t\r \u00C2\u00A0 8.5\t\r \u00C2\u00A08.4\t\r \u00C2\u00A09.1\t\r \u00C2\u00A05.2\t\r \u00C2\u00A0 8.6\t\r \u00C2\u00A07.8\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A05.5\t\r \u00C2\u00A03.7\t\r \u00C2\u00A0 0\t\r \u00C2\u00A02\t\r \u00C2\u00A0 4\t\r \u00C2\u00A06\t\r \u00C2\u00A0 8\t\r \u00C2\u00A010\t\r \u00C2\u00A0 12\t\r \u00C2\u00A014\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 1988\t\r \u00C2\u00A0 1989\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 1991\t\r \u00C2\u00A0 1992\t\r \u00C2\u00A0 1993\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 1995\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 1997\t\r \u00C2\u00A0 1998\t\r \u00C2\u00A0 1999\t\r \u00C2\u00A0 2000\t\r \u00C2\u00A0 2001\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 2003\t\r \u00C2\u00A0 2004\t\r \u00C2\u00A0 2005\t\r \u00C2\u00A0 2006\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 2009\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 2011\t\r \u00C2\u00A0 Inciden ce\t\r \u00C2\u00A0(/m illion/y ear)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Mean:\t\r \u00C2\u00A07.8\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Mean:\t\r \u00C2\u00A07.2\t\r \u00C2\u00A0Current\t\r \u00C2\u00A0report\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test Mean:\t\r \u00C2\u00A05.7\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 69\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A024b.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A01993\t\r \u00C2\u00A0using\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0 criteria\t\r \u00C2\u00A0only.\t\r \u00C2\u00A0Error\t\r \u00C2\u00A0bars\t\r \u00C2\u00A0represent\t\r \u00C2\u00A095%\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0intervals\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A025.\t\r \u00C2\u00A0Global\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time\t\r \u00C2\u00A0and\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0 comparative\t\r \u00C2\u00A0purposes,\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0after\t\r \u00C2\u00A01993\t\r \u00C2\u00A0include\t\r \u00C2\u00A0only\t\r \u00C2\u00A0diagnoses\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0 genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 5.9\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 7.4\t\r \u00C2\u00A0 7.5\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A0 7.8\t\r \u00C2\u00A0 6.0\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 6.1\t\r \u00C2\u00A0 7.8\t\r \u00C2\u00A0 8.5\t\r \u00C2\u00A0 8.2\t\r \u00C2\u00A0 8.8\t\r \u00C2\u00A0 4.7\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0 7.5\t\r \u00C2\u00A0 6.1\t\r \u00C2\u00A0 4.9\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 0\t\r \u00C2\u00A02\t\r \u00C2\u00A0 4\t\r \u00C2\u00A06\t\r \u00C2\u00A0 8\t\r \u00C2\u00A010\t\r \u00C2\u00A0 12\t\r \u00C2\u00A014\t\r \u00C2\u00A0 1993\t\r \u00C2\u00A01994\t\r \u00C2\u00A01995\t\r \u00C2\u00A01996\t\r \u00C2\u00A01997\t\r \u00C2\u00A01998\t\r \u00C2\u00A01999\t\r \u00C2\u00A02000\t\r \u00C2\u00A02001\t\r \u00C2\u00A02002\t\r \u00C2\u00A02003\t\r \u00C2\u00A02004\t\r \u00C2\u00A02005\t\r \u00C2\u00A02006\t\r \u00C2\u00A02007\t\r \u00C2\u00A02008\t\r \u00C2\u00A02009\t\r \u00C2\u00A02010\t\r \u00C2\u00A02011\t\r \u00C2\u00A0 Inciden ce\t\r \u00C2\u00A0(/m illion/y ear)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Mean:\t\r \u00C2\u00A06.9\t\r \u00C2\u00A0(Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Mean:\t\r \u00C2\u00A06.5\t\r \u00C2\u00A0Current\t\r \u00C2\u00A0report\t\r \u00C2\u00A0 3.0\t\r \u00C2\u00A0 4.0\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 4.7\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 4.4\t\r \u00C2\u00A0 6.1\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 0\t\r \u00C2\u00A01\t\r \u00C2\u00A02\t\r \u00C2\u00A0 3\t\r \u00C2\u00A04\t\r \u00C2\u00A05\t\r \u00C2\u00A0 6\t\r \u00C2\u00A07\t\r \u00C2\u00A08\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 1989\t\r \u00C2\u00A0 2000\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 2011\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0 USA\t\r \u00C2\u00A0 Canada\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0 Spain\t\r \u00C2\u00A0 Taiwan\t\r \u00C2\u00A0 Greece\t\r \u00C2\u00A0 UK\t\r \u00C2\u00A0 Canada\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0 Inciden ce\t\r \u00C2\u00A0(/m illion/y ear)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0Direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0available \t\r \u00C2\u00A0 <1993:\t\r \u00C2\u00A0prior\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test \t\r \u00C2\u00A0 70\t\r \u00C2\u00A0 Sources\t\r \u00C2\u00A0of\t\r \u00C2\u00A0error:\t\r \u00C2\u00A0caveats\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0are\t\r \u00C2\u00A0related\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0accuracy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0information\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0in\t\r \u00C2\u00A0patients\u00E2\u0080\u0099\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0files.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Some\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0calculation\t\r \u00C2\u00A0because\t\r \u00C2\u00A0they\t\r \u00C2\u00A0had\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0and\t\r \u00C2\u00A0were\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0become\t\r \u00C2\u00A0symptomatic\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Other\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0who\t\r \u00C2\u00A0had\t\r \u00C2\u00A0never\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0the\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0(or\t\r \u00C2\u00A0their\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available)\t\r \u00C2\u00A0but\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0also\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0become\t\r \u00C2\u00A0symptomatic\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Year\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0was\t\r \u00C2\u00A0obtained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0notes\t\r \u00C2\u00A0during\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0and\t\r \u00C2\u00A0was,\t\r \u00C2\u00A0at\t\r \u00C2\u00A0times,\t\r \u00C2\u00A0expressed\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0time\t\r \u00C2\u00A0frame\t\r \u00C2\u00A0of\t\r \u00C2\u00A0several\t\r \u00C2\u00A0years\t\r \u00C2\u00A0rather\t\r \u00C2\u00A0than\t\r \u00C2\u00A0a\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0date.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Under\t\r \u00C2\u00A0such\t\r \u00C2\u00A0circumstances,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0median\t\r \u00C2\u00A0year\t\r \u00C2\u00A0was\t\r \u00C2\u00A0recorded.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0this\t\r \u00C2\u00A0reason,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0annual\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0may\t\r \u00C2\u00A0lack\t\r \u00C2\u00A0accuracy.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Additionally,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0may\t\r \u00C2\u00A0me\t\r \u00C2\u00A0underestimated.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0calculation,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0information\t\r \u00C2\u00A0(or\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test)\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0the\t\r \u00C2\u00A0date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0information\t\r \u00C2\u00A0was\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0174\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0(patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0outside\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r 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\u00C2\u00A0in\t\r \u00C2\u00A0many\t\r \u00C2\u00A0cases,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0individual\u00E2\u0080\u0099s\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0developing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0decreases\t\r \u00C2\u00A0for\t\r \u00C2\u00A0every\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0year\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0is\t\r \u00C2\u00A0alive\t\r \u00C2\u00A0and\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0free.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0demographic\t\r \u00C2\u00A0information\t\r \u00C2\u00A0was\t\r \u00C2\u00A0only\t\r \u00C2\u00A0available\t\r \u00C2\u00A0for\t\r \u00C2\u00A0a\t\r \u00C2\u00A0small\t\r \u00C2\u00A0portion\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0age-\u00C2\u00AD\u00E2\u0080\u0090specific\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0could\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0acquired.\t\r \u00C2\u00A0Three\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0comparisons\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0findings\t\r \u00C2\u00A0obtained\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study.\t\r \u00C2\u00A0\t\r \u00C2\u00A0One\t\r \u00C2\u00A0study\t\r \u00C2\u00A0was\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0(Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000)\t\r \u00C2\u00A0one\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria,\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0last,\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0(Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0other\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0our\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Taylor\t\r \u00C2\u00A01994,\t\r \u00C2\u00A0Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999,\t\r \u00C2\u00A0Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000,\t\r \u00C2\u00A0Goizet\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Dutch,\t\r \u00C2\u00A0Australian\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Irish\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0along\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study\t\r \u00C2\u00A0from\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0have\t\r \u00C2\u00A0all\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0directly\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0hand.\t\r \u00C2\u00A0\t\r \u00C2\u00A0When\t\r \u00C2\u00A0comparing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0proportion\t\r \u00C2\u00A0to\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0between\t\r \u00C2\u00A0these\t\r \u00C2\u00A0studies,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Dutch\t\r \u00C2\u00A0study\t\r \u00C2\u00A0has\t\r \u00C2\u00A0the\t\r \u00C2\u00A0largest\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0relative\t\r \u00C2\u00A0to\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0being\t\r \u00C2\u00A0five\t\r \u00C2\u00A0times\t\r \u00C2\u00A0the\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A021).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Australian\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Irish\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0follow\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A04.2\t\r \u00C2\u00A0times\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A026).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0present\t\r \u00C2\u00A0study\t\r \u00C2\u00A0from\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0appears\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0the\t\r \u00C2\u00A0lowest\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0relative\t\r \u00C2\u00A0to\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0only\t\r \u00C2\u00A03.6\t\r \u00C2\u00A0times\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0(Figure\t\r \u00C2\u00A026).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0relative\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 72\t\r \u00C2\u00A0 population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Dutch\t\r \u00C2\u00A0study\t\r \u00C2\u00A0is\t\r \u00C2\u00A0keeping\t\r \u00C2\u00A0with\t\r \u00C2\u00A0Conneally\u00E2\u0080\u0099s\t\r \u00C2\u00A0theory\t\r \u00C2\u00A0of\t\r \u00C2\u00A01:5\t\r \u00C2\u00A0affected:\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0ratio\t\r \u00C2\u00A0(Conneally,\t\r \u00C2\u00A01984).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Australian\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Irish\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0are\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0than\t\r \u00C2\u00A0would\t\r \u00C2\u00A0be\t\r \u00C2\u00A0expected\t\r \u00C2\u00A0by\t\r 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\u00C2\u00A0one\t\r \u00C2\u00A0of\t\r \u00C2\u00A0two\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0factors:\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0or\t\r \u00C2\u00A0an\t\r \u00C2\u00A0underestimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0In\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0a\t\r \u00C2\u00A0high\t\r \u00C2\u00A0degree\t\r \u00C2\u00A0of\t\r \u00C2\u00A0rigor\t\r \u00C2\u00A0was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0that\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Multiple\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0including\t\r \u00C2\u00A0follow-\u00C2\u00AD\u00E2\u0080\u0090up\t\r \u00C2\u00A0on\t\r \u00C2\u00A0secondary\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0were\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0process.\t\r \u00C2\u00A0\t\r \u00C2\u00A0All\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0included\t\r \u00C2\u00A0had\t\r \u00C2\u00A0been\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0majority\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0were\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A02).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Conversely,\t\r \u00C2\u00A0fewer\t\r \u00C2\u00A0avenues\t\r \u00C2\u00A0were\t\r \u00C2\u00A0available\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0either\t\r \u00C2\u00A0active\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0and/or\t\r \u00C2\u00A0counselling\t\r \u00C2\u00A0or\t\r \u00C2\u00A0they\t\r \u00C2\u00A0were\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0included\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0of\t\r \u00C2\u00A0active\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r 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\u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Similar\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0population,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0may\t\r \u00C2\u00A0either\t\r \u00C2\u00A0be\t\r \u00C2\u00A0a\t\r \u00C2\u00A0reflection\t\r \u00C2\u00A0of\t\r \u00C2\u00A0under-\u00C2\u00AD\u00E2\u0080\u0090ascertainment\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0as\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r 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\u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0outside\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0and\t\r \u00C2\u00A0as\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0were\t\r \u00C2\u00A0only\t\r \u00C2\u00A0available\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0region\t\r \u00C2\u00A0is\t\r \u00C2\u00A0likely\t\r \u00C2\u00A0more\t\r \u00C2\u00A0thorough\t\r \u00C2\u00A0than\t\r \u00C2\u00A0that\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0regions.\t\r \u00C2\u00A0When\t\r 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\u00C2\u00A0Victoria\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0 Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0 Canada\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0 Popula tion\t\r \u00C2\u00A0(/ 100,00 0)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0Population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0(/100,000)\t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A026.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0ratio\t\r 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\u00C2\u00A0present\t\r \u00C2\u00A0study\t\r \u00C2\u00A0than\t\r \u00C2\u00A0was\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0account\t\r \u00C2\u00A0of\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0a\t\r \u00C2\u00A0result,\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0conduct\t\r \u00C2\u00A0a\t\r \u00C2\u00A0meaningful\t\r \u00C2\u00A0comparison\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0two\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0and\t\r \u00C2\u00A0in\t\r \u00C2\u00A0turn\t\r \u00C2\u00A0understand\t\r \u00C2\u00A0trends\t\r \u00C2\u00A0in\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province,\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0was\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090calculated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0each\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period\t\r \u00C2\u00A0using\t\r \u00C2\u00A0both\t\r \u00C2\u00A0the\t\r \u00C2\u00A0original\t\r \u00C2\u00A0equation\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0revised\t\r \u00C2\u00A0equation.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Additionally,\t\r \u00C2\u00A0three\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0were\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0to\t\r \u00C2\u00A0both\t\r \u00C2\u00A0equations\t\r \u00C2\u00A0(Table\t\r \u00C2\u00A012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0appears\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0has\t\r \u00C2\u00A0declined\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0since\t\r \u00C2\u00A02000.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Using\t\r \u00C2\u00A0all\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0both\t\r \u00C2\u00A0equations\t\r \u00C2\u00A0for\t\r \u00C2\u00A0uptake,\t\r \u00C2\u00A0all\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902012\t\r \u00C2\u00A0values\t\r \u00C2\u00A0remain\t\r \u00C2\u00A0lower\t\r \u00C2\u00A0than\t\r \u00C2\u00A0those\t\r \u00C2\u00A0from\t\r \u00C2\u00A01987-\u00C2\u00AD\u00E2\u0080\u00902000.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0decline\t\r \u00C2\u00A0in\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0related\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0enthusiasm\t\r \u00C2\u00A0surrounding\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0during\t\r \u00C2\u00A0its\t\r \u00C2\u00A0beginning\t\r \u00C2\u00A0stages\t\r \u00C2\u00A0and\t\r \u00C2\u00A0potentially\t\r \u00C2\u00A0higher\t\r \u00C2\u00A0hopes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0development\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0treatment\t\r \u00C2\u00A0during\t\r \u00C2\u00A0these\t\r \u00C2\u00A0first\t\r \u00C2\u00A0ten\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0availability.\t\r \u00C2\u00A0\t\r \u00C2\u00A0From\t\r \u00C2\u00A0Table\t\r \u00C2\u00A012,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0noted\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0results\t\r \u00C2\u00A0differ\t\r \u00C2\u00A0considerably\t\r \u00C2\u00A0between\t\r \u00C2\u00A0equations.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0supports\t\r \u00C2\u00A0the\t\r \u00C2\u00A0notion\t\r \u00C2\u00A0that\t\r \u00C2\u00A0a\t\r \u00C2\u00A0standard\t\r \u00C2\u00A0method\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0adopted\t\r \u00C2\u00A0when\t\r \u00C2\u00A0considering\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Without\t\r \u00C2\u00A0a\t\r \u00C2\u00A0standardized\t\r \u00C2\u00A0method,\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0cannot\t\r \u00C2\u00A0be\t\r \u00C2\u00A0meaningfully\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0between\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0and\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Secondly,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0results\t\r \u00C2\u00A0differ\t\r \u00C2\u00A0considerably\t\r \u00C2\u00A0when\t\r \u00C2\u00A0different\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0are\t\r \u00C2\u00A0used\t\r \u00C2\u00A0to\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0most\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0incorporate\t\r \u00C2\u00A0an\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0population\t\r \u00C2\u00A0by\t\r \u00C2\u00A0multiplying\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0by\t\r \u00C2\u00A04.2\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009,\t\r \u00C2\u00A0Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0or\t\r \u00C2\u00A0by\t\r \u00C2\u00A05\t\r \u00C2\u00A0(Conneally\t\r \u00C2\u00A01984,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003),\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0that\t\r \u00C2\u00A0an\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0available.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Underestimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0render\t\r \u00C2\u00A0overestimates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0uptake.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0therefore\t\r \u00C2\u00A0important\t\r \u00C2\u00A0that\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0wishing\t\r \u00C2\u00A0to\t\r \u00C2\u00A0assess\t\r \u00C2\u00A0the\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0using\t\r \u00C2\u00A0theoretical\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 76\t\r \u00C2\u00A0 methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0estimating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0first\t\r \u00C2\u00A0ensure\t\r \u00C2\u00A0that\t\r \u00C2\u00A0a\t\r \u00C2\u00A0thorough\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0ascertainment\t\r \u00C2\u00A0study\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0conducted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0their\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Table\t\r \u00C2\u00A012.\t\r \u00C2\u00A0Comparing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0over\t\r \u00C2\u00A0two\t\r \u00C2\u00A0study\t\r \u00C2\u00A0periods.\t\r \u00C2\u00A0 Three\t\r \u00C2\u00A0separate\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0are\t\r \u00C2\u00A0applied\t\r \u00C2\u00A0while\t\r \u00C2\u00A0two\t\r \u00C2\u00A0 separate\t\r \u00C2\u00A0methods\t\r \u00C2\u00A0of\t\r \u00C2\u00A0calculating\t\r \u00C2\u00A0the\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0are\t\r \u00C2\u00A0applied.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 UPTAKE\t\r \u00C2\u00A0 Study\t\r \u00C2\u00A0period\t\r \u00C2\u00A0 Pop.\t\r \u00C2\u00A0At\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0 P\t\r \u00C2\u00A0 D\t\r \u00C2\u00A0 Method\t\r \u00C2\u00A01\t\r \u00C2\u00A0 D/P\t\r \u00C2\u00A0 Method\t\r \u00C2\u00A02\t\r \u00C2\u00A0 D/P+(P*t/18.8)\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Method\t\r \u00C2\u00A01:\t\r \u00C2\u00A0Theoretical\t\r \u00C2\u00A0\t\r \u00C2\u00A0(prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A08.4/100,000)\t\r \u00C2\u00A0 1987-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0 1268\t\r \u00C2\u00A0 672\t\r \u00C2\u00A0 354\t\r \u00C2\u00A0 53%\t\r \u00C2\u00A0 32%\t\r \u00C2\u00A0 2000-\u00C2\u00AD\u00E2\u0080\u00902012\t\r \u00C2\u00A0 1626\t\r \u00C2\u00A0 976\t\r \u00C2\u00A0 280\t\r \u00C2\u00A0 29%\t\r \u00C2\u00A0 18%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Method\t\r \u00C2\u00A02:\t\r \u00C2\u00A0Theoretical\t\r \u00C2\u00A0\t\r \u00C2\u00A0(prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A013.9/100,000)\t\r \u00C2\u00A0 1987-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 1049.4\t\r \u00C2\u00A0 354\t\r \u00C2\u00A0 34%\t\r \u00C2\u00A0 20%\t\r \u00C2\u00A0 2000-\u00C2\u00AD\u00E2\u0080\u00902012\t\r \u00C2\u00A0 2536\t\r \u00C2\u00A0 1522\t\r \u00C2\u00A0 280\t\r \u00C2\u00A0 18%\t\r \u00C2\u00A0 11%\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Method\t\r \u00C2\u00A03:\t\r \u00C2\u00A0Empirical\t\r \u00C2\u00A0data\t\r \u00C2\u00A0 1987-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0 1758\t\r \u00C2\u00A0 932\t\r \u00C2\u00A0 354\t\r \u00C2\u00A0 38%\t\r \u00C2\u00A0 23%\t\r \u00C2\u00A0 2000-\u00C2\u00AD\u00E2\u0080\u00902012\t\r \u00C2\u00A0 2252\t\r \u00C2\u00A0 1351\t\r \u00C2\u00A0 280\t\r \u00C2\u00A0 21%\t\r \u00C2\u00A0 13%\t\r \u00C2\u00A0 D=\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0tests\t\r \u00C2\u00A0performed;\t\r \u00C2\u00A0P=\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0eligible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0(age\t\r \u00C2\u00A018\t\r \u00C2\u00A0or\t\r \u00C2\u00A0above);\t\r \u00C2\u00A0t=number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0years\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period;\t\r \u00C2\u00A018.8=\t\r \u00C2\u00A0average\t\r \u00C2\u00A0duration\t\r \u00C2\u00A0of\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0(HD\t\r \u00C2\u00A0symptoms)\t\r \u00C2\u00A0(Tassicker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 77\t\r \u00C2\u00A0 Direct\t\r \u00C2\u00A0and\t\r \u00C2\u00A0immediate\t\r \u00C2\u00A0implications\t\r \u00C2\u00A0This\t\r \u00C2\u00A0study\t\r \u00C2\u00A0comprises\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0(HD)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0(BC).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Generation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0services\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0has\t\r \u00C2\u00A0occurred,\t\r \u00C2\u00A0thus\t\r \u00C2\u00A0far,\t\r \u00C2\u00A0without\t\r \u00C2\u00A0data\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence,\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0geographical\t\r \u00C2\u00A0distribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Without\t\r \u00C2\u00A0these\t\r \u00C2\u00A0figures,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0difficult\t\r \u00C2\u00A0to\t\r \u00C2\u00A0produce\t\r \u00C2\u00A0a\t\r \u00C2\u00A0compelling\t\r \u00C2\u00A0argument\t\r \u00C2\u00A0to\t\r \u00C2\u00A0justify\t\r \u00C2\u00A0the\t\r \u00C2\u00A0allocation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0public\t\r \u00C2\u00A0resources\t\r \u00C2\u00A0to\t\r \u00C2\u00A0aid\t\r \u00C2\u00A0in\t\r \u00C2\u00A0supporting\t\r \u00C2\u00A0these\t\r \u00C2\u00A0services.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Moreover,\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0extrapolated\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0have\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0underestimates\t\r \u00C2\u00A0(Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003).\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0study\t\r \u00C2\u00A0provides\t\r \u00C2\u00A0the\t\r \u00C2\u00A0baseline\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0that\t\r \u00C2\u00A0are\t\r \u00C2\u00A0essential\t\r \u00C2\u00A0for\t\r \u00C2\u00A0an\t\r \u00C2\u00A0accurate\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0service\t\r \u00C2\u00A0and\t\r \u00C2\u00A0care\t\r \u00C2\u00A0requirements\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0Through\t\r \u00C2\u00A0our\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0surveys,\t\r \u00C2\u00A0we\t\r \u00C2\u00A0have\t\r \u00C2\u00A0identified\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0regions\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province\t\r \u00C2\u00A0where\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0are\t\r \u00C2\u00A0numerous.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0obtained\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0cared\t\r \u00C2\u00A0for\t\r \u00C2\u00A0by\t\r \u00C2\u00A0each\t\r \u00C2\u00A0responding\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0represents\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0that\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0useful\t\r \u00C2\u00A0for\t\r \u00C2\u00A0targeting\t\r \u00C2\u00A0new\t\r \u00C2\u00A0programs.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0example,\t\r \u00C2\u00A0one\t\r \u00C2\u00A0general\t\r \u00C2\u00A0practitioner\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alice\t\r \u00C2\u00A0indicated\t\r \u00C2\u00A0that\t\r \u00C2\u00A0he\t\r \u00C2\u00A0cares\t\r \u00C2\u00A0for\t\r \u00C2\u00A0ten\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A0none\t\r \u00C2\u00A0of\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH.\t\r \u00C2\u00A0\t\r \u00C2\u00A0No\t\r \u00C2\u00A0neurologist\t\r \u00C2\u00A0works\t\r \u00C2\u00A0in\t\r \u00C2\u00A0or\t\r \u00C2\u00A0near\t\r \u00C2\u00A0to\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alice,\t\r \u00C2\u00A0indicating\t\r \u00C2\u00A0this\t\r \u00C2\u00A0GP\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0the\t\r \u00C2\u00A0sole\t\r \u00C2\u00A0care\t\r \u00C2\u00A0provider\t\r \u00C2\u00A0for\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0GP\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0suitable\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0key\t\r \u00C2\u00A0player\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0pilot\t\r \u00C2\u00A0project\t\r \u00C2\u00A0to\t\r \u00C2\u00A0act\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0\u00E2\u0080\u009Cremote\t\r \u00C2\u00A0representative\u00E2\u0080\u009D\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0resources\t\r \u00C2\u00A0are\t\r \u00C2\u00A0scarce\t\r \u00C2\u00A0and\t\r \u00C2\u00A0public\t\r \u00C2\u00A0funding\t\r \u00C2\u00A0involvement\t\r \u00C2\u00A0absent\t\r \u00C2\u00A0in\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province,\t\r \u00C2\u00A0recognizing\t\r \u00C2\u00A0professionals\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0already\t\r \u00C2\u00A0involved\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0as\t\r \u00C2\u00A0remote\t\r \u00C2\u00A0representatives,\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0an\t\r \u00C2\u00A0appropriate\t\r \u00C2\u00A0first\t\r \u00C2\u00A0step\t\r \u00C2\u00A0towards\t\r \u00C2\u00A0strengthening\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0resource\t\r \u00C2\u00A0infrastructure\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0province.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further,\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0population\t\r \u00C2\u00A0of\t\r \u00C2\u00A0only\t\r \u00C2\u00A0821\t\r \u00C2\u00A0(BC\t\r \u00C2\u00A0Stats,\t\r \u00C2\u00A02011),\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A01/82\t\r \u00C2\u00A0people\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alice\t\r \u00C2\u00A0may\t\r \u00C2\u00A0suffer\t\r \u00C2\u00A0from\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0this\t\r \u00C2\u00A0is\t\r \u00C2\u00A0clearly\t\r \u00C2\u00A0the\t\r \u00C2\u00A0highest\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0\t\r \u00C2\u00A0If\t\r \u00C2\u00A0we\t\r \u00C2\u00A0extrapolate\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0estimates\t\r \u00C2\u00A0obtained\t\r \u00C2\u00A0for\t\r \u00C2\u00A0BC,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0suggests\t\r \u00C2\u00A0that\t\r \u00C2\u00A0countrywide,\t\r \u00C2\u00A0there\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A04,700\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A04,586-\u00C2\u00AD\u00E2\u0080\u00904,862)\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A017,000\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0presently\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A0250\t\r \u00C2\u00A0new\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0this\t\r \u00C2\u00A0year.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Further,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0implies\t\r \u00C2\u00A0that\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0United\t\r \u00C2\u00A0States,\t\r \u00C2\u00A0there\t\r \u00C2\u00A0may\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A042,000\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A042,225-\u00C2\u00AD\u00E2\u0080\u009042,945)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 78\t\r \u00C2\u00A0 individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0153,000\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0presently\t\r \u00C2\u00A0living\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0States,\t\r \u00C2\u00A0and\t\r 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\u00C2\u00A0study\t\r \u00C2\u00A0comprised\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0modern\t\r \u00C2\u00A0and\t\r \u00C2\u00A0comprehensive\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0ranging\t\r \u00C2\u00A0from\t\r \u00C2\u00A012.5-\u00C2\u00AD\u00E2\u0080\u009014.9/100,000\t\r \u00C2\u00A0(1/8,000-\u00C2\u00AD\u00E2\u0080\u00901/6,711)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A011.5-\u00C2\u00AD\u00E2\u0080\u009016.0\t\r \u00C2\u00A0[1/8,696-\u00C2\u00AD\u00E2\u0080\u00901/6,250])\t\r \u00C2\u00A0was\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0general\t\r \u00C2\u00A0population\t\r \u00C2\u00A0and\t\r \u00C2\u00A0approximately\t\r \u00C2\u00A017.20/100,000\t\r \u00C2\u00A0(1/5,814)\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A017.19-\u00C2\u00AD\u00E2\u0080\u009017.20)\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Caucasians.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0highest\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0since\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test\t\r \u00C2\u00A0in\t\r \u00C2\u00A01993\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0twice\t\r \u00C2\u00A0as\t\r \u00C2\u00A0high\t\r \u00C2\u00A0as\t\r \u00C2\u00A0that\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0(Shokeir,\t\r \u00C2\u00A01975).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0was\t\r \u00C2\u00A0between\t\r \u00C2\u00A06.9-\u00C2\u00AD\u00E2\u0080\u00907.2\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(95%\t\r \u00C2\u00A0CI:\t\r \u00C2\u00A04.4-\u00C2\u00AD\u00E2\u0080\u00907.9)\t\r \u00C2\u00A0between\t\r \u00C2\u00A0the\t\r \u00C2\u00A0years\t\r \u00C2\u00A02000\t\r \u00C2\u00A0and\t\r \u00C2\u00A02011\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0very\t\r \u00C2\u00A0close\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0for\t\r \u00C2\u00A01996-\u00C2\u00AD\u00E2\u0080\u00901999\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A06.9\t\r \u00C2\u00A0per\t\r \u00C2\u00A0million/year\t\r \u00C2\u00A0(Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001).\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0was\t\r \u00C2\u00A0found\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A094.0/100,000\t\r \u00C2\u00A0or\t\r \u00C2\u00A01/1,073.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0study\t\r \u00C2\u00A0was\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0to\t\r \u00C2\u00A0estimate\t\r \u00C2\u00A0both\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A050%\t\r \u00C2\u00A0a\t\r \u00C2\u00A0priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A025%\t\r \u00C2\u00A0a\t\r \u00C2\u00A0priori\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0and\t\r \u00C2\u00A0was\t\r \u00C2\u00A0only\t\r \u00C2\u00A0the\t\r \u00C2\u00A0fourth\t\r \u00C2\u00A0study\t\r \u00C2\u00A0to\t\r \u00C2\u00A0calculate\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r 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\u00C2\u00A0confident\t\r \u00C2\u00A0results\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date.\t\r \u00C2\u00A0\t\r \u00C2\u00A0It\t\r \u00C2\u00A0is\t\r \u00C2\u00A0also\t\r \u00C2\u00A0the\t\r \u00C2\u00A0first\t\r \u00C2\u00A0study\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0ethnicity\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0these\t\r \u00C2\u00A0reasons,\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study\t\r \u00C2\u00A0may\t\r \u00C2\u00A0set\t\r \u00C2\u00A0a\t\r \u00C2\u00A0precedent\t\r \u00C2\u00A0for\t\r \u00C2\u00A0future\t\r \u00C2\u00A0work\t\r \u00C2\u00A0and\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0compared\t\r \u00C2\u00A0with\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0to\t\r \u00C2\u00A0future\t\r \u00C2\u00A0findings\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0gain\t\r \u00C2\u00A0a\t\r \u00C2\u00A0greater\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0across\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0regions,\t\r \u00C2\u00A0between\t\r \u00C2\u00A0ethnicities\t\r \u00C2\u00A0and\t\r \u00C2\u00A0over\t\r \u00C2\u00A0time.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 80\t\r \u00C2\u00A0 Future\t\r \u00C2\u00A0direction\t\r \u00C2\u00A0\t\r \u00C2\u00A0 When\t\r \u00C2\u00A0ascertaining\t\r \u00C2\u00A0symptomatic\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A0only\t\r \u00C2\u00A0minimum\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0can\t\r \u00C2\u00A0be\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0as\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0may\t\r \u00C2\u00A0not\t\r \u00C2\u00A0seek\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0attention\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0study\t\r \u00C2\u00A0group\t\r \u00C2\u00A0may\t\r \u00C2\u00A0fail\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0the\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0records\t\r \u00C2\u00A0for\t\r \u00C2\u00A0every\t\r \u00C2\u00A0patient.\t\r \u00C2\u00A0\t\r \u00C2\u00A0These\t\r \u00C2\u00A0limitations\t\r \u00C2\u00A0become\t\r \u00C2\u00A0more\t\r \u00C2\u00A0apparent\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0shorter\t\r \u00C2\u00A0study\t\r \u00C2\u00A0period.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0of\t\r \u00C2\u00A0intermediate\t\r \u00C2\u00A0allele\t\r \u00C2\u00A0(IA),\t\r \u00C2\u00A0reduced\t\r \u00C2\u00A0penetrance\t\r \u00C2\u00A0(RP)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0full\t\r \u00C2\u00A0penetrance\t\r \u00C2\u00A0(FP)\t\r \u00C2\u00A0alleles\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0general\t\r \u00C2\u00A0population\t\r \u00C2\u00A0should\t\r \u00C2\u00A0be\t\r \u00C2\u00A0assessed.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0information\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0for\t\r \u00C2\u00A0an\t\r \u00C2\u00A0understanding\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0true\t\r \u00C2\u00A0heterozygousity\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0(Harper,\t\r \u00C2\u00A01991)\t\r \u00C2\u00A0and\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predicting\t\r \u00C2\u00A0future\t\r \u00C2\u00A0patterns\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0A\t\r \u00C2\u00A0large\t\r \u00C2\u00A0random\t\r \u00C2\u00A0sample\t\r \u00C2\u00A0population\t\r \u00C2\u00A0would\t\r \u00C2\u00A0be\t\r \u00C2\u00A0required\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0such\t\r \u00C2\u00A0as\t\r \u00C2\u00A0blood\t\r \u00C2\u00A0spot\t\r \u00C2\u00A0cards9\t\r \u00C2\u00A0from\t\r \u00C2\u00A0local\t\r \u00C2\u00A0hospitals\t\r \u00C2\u00A0or\t\r \u00C2\u00A0random\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0samples\t\r \u00C2\u00A0from\t\r \u00C2\u00A0unrelated\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0general\t\r \u00C2\u00A0population.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Additionally,\t\r \u00C2\u00A0an\t\r \u00C2\u00A0investigation\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0in\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0of\t\r \u00C2\u00A0nursing\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0would\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0further\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Dementia\t\r \u00C2\u00A0is\t\r \u00C2\u00A0widespread\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada;\t\r \u00C2\u00A08%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0over\t\r \u00C2\u00A065\t\r \u00C2\u00A0and\t\r \u00C2\u00A035%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0those\t\r \u00C2\u00A0over\t\r \u00C2\u00A085\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0age\t\r \u00C2\u00A0suffer\t\r \u00C2\u00A0from\t\r \u00C2\u00A0dementia\t\r \u00C2\u00A0(Long\t\r \u00C2\u00A0term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0expected\t\r \u00C2\u00A0to\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0significantly\t\r \u00C2\u00A0more\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0aging\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0baby\t\r \u00C2\u00A0boom\t\r \u00C2\u00A0cohort\t\r \u00C2\u00A0(Statistics\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Although\t\r \u00C2\u00A0commonly\t\r \u00C2\u00A0characterized\t\r \u00C2\u00A0as\t\r \u00C2\u00A0having\t\r \u00C2\u00A0a\t\r \u00C2\u00A0distinct\t\r \u00C2\u00A0phenotype\t\r \u00C2\u00A0(Walker,\t\r \u00C2\u00A02007),\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0can\t\r \u00C2\u00A0vary\t\r \u00C2\u00A0quite\t\r \u00C2\u00A0dramatically\t\r \u00C2\u00A0between\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0(personal\t\r \u00C2\u00A0correspondence).\t\r \u00C2\u00A0\t\r \u00C2\u00A0Very\t\r \u00C2\u00A0late\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0may\t\r \u00C2\u00A0exist\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0form\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0dementia-\u00C2\u00AD\u00E2\u0080\u0090like\t\r \u00C2\u00A0phenotype\t\r \u00C2\u00A0without\t\r \u00C2\u00A0chorea,\t\r \u00C2\u00A0leading\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0possibility\t\r \u00C2\u00A0of\t\r \u00C2\u00A0missed\t\r \u00C2\u00A0cases.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0elderly\t\r \u00C2\u00A0population\t\r \u00C2\u00A0would\t\r \u00C2\u00A0allow\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0opportunity\t\r \u00C2\u00A0to\t\r \u00C2\u00A0detect\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0dementia-\u00C2\u00AD\u00E2\u0080\u0090like\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0would\t\r \u00C2\u00A0in\t\r \u00C2\u00A0turn,\t\r \u00C2\u00A0further\t\r \u00C2\u00A0increase\t\r \u00C2\u00A0the\t\r \u00C2\u00A0estimated\t\r \u00C2\u00A0prevalence.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0mean\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0size\t\r \u00C2\u00A0of\t\r \u00C2\u00A0control\t\r \u00C2\u00A0chromosomes\t\r \u00C2\u00A0across\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0varying\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0is\t\r \u00C2\u00A0required.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Warby\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0(2011)\t\r \u00C2\u00A0suggest\t\r \u00C2\u00A0that\t\r \u00C2\u00A0the\t\r \u00C2\u00A0difference\t\r \u00C2\u00A0in\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0observed\t\r \u00C2\u00A0between\t\r \u00C2\u00A0ethnicities\t\r \u00C2\u00A0is\t\r \u00C2\u00A0attributable\t\r \u00C2\u00A0in\t\r \u00C2\u00A0part\t\r \u00C2\u00A0to\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A09\t\r \u00C2\u00A0Blood\t\r \u00C2\u00A0spot\t\r \u00C2\u00A0cards\t\r \u00C2\u00A0are\t\r \u00C2\u00A0cards\t\r \u00C2\u00A0that\t\r \u00C2\u00A0contain\t\r \u00C2\u00A0small\t\r \u00C2\u00A0blood\t\r \u00C2\u00A0samples\t\r \u00C2\u00A0for\t\r \u00C2\u00A0every\t\r \u00C2\u00A0newborn\t\r \u00C2\u00A0baby\t\r \u00C2\u00A0in\t\r \u00C2\u00A0hospitals\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0part\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0newborn\t\r \u00C2\u00A0screening\t\r \u00C2\u00A0program.\t\r \u00C2\u00A0Newborn\t\r \u00C2\u00A0Screening\t\r \u00C2\u00A0can\t\r \u00C2\u00A0identify\t\r \u00C2\u00A0babies\t\r \u00C2\u00A0who\t\r \u00C2\u00A0may\t\r \u00C2\u00A0have\t\r \u00C2\u00A0one\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0treatable\t\r \u00C2\u00A0disorders.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 81\t\r \u00C2\u00A0 specific\t\r \u00C2\u00A0haplotypes.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Falush\t\r \u00C2\u00A0(2009)\t\r \u00C2\u00A0suggested\t\r \u00C2\u00A0the\t\r \u00C2\u00A0cross-\u00C2\u00AD\u00E2\u0080\u0090ethnic\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r 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\u00C2\u00A0clinic\t\r \u00C2\u00A0already\t\r \u00C2\u00A0composes\t\r \u00C2\u00A0the\t\r \u00C2\u00A0framework\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0team;\t\r \u00C2\u00A0a\t\r \u00C2\u00A0multidisciplinary\t\r \u00C2\u00A0team\t\r \u00C2\u00A0with\t\r \u00C2\u00A0specialty\t\r \u00C2\u00A0in\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0provides\t\r \u00C2\u00A0care\t\r \u00C2\u00A0to\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinic.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0addition\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0register\t\r \u00C2\u00A0manager\t\r \u00C2\u00A0would\t\r \u00C2\u00A0largely\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0this\t\r \u00C2\u00A0team.\t\r \u00C2\u00A0\t\r \u00C2\u00A02)\t\r \u00C2\u00A0Long-\u00C2\u00AD\u00E2\u0080\u0090term,\t\r \u00C2\u00A0stable\t\r \u00C2\u00A0funding\t\r 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\u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province,\t\r \u00C2\u00A0ethical\t\r \u00C2\u00A0approval\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0acquired\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0addition\t\r \u00C2\u00A0of\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0demographic\t\r \u00C2\u00A0information\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0register\t\r \u00C2\u00A0from\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0were\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0project\t\r \u00C2\u00A0from\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0outside\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0VHG\t\r \u00C2\u00A0(nursing\t\r \u00C2\u00A0homes,\t\r \u00C2\u00A0neurologist\t\r \u00C2\u00A0and\t\r \u00C2\u00A0GP\t\r \u00C2\u00A0records,\t\r \u00C2\u00A0family\t\r \u00C2\u00A0surveys).\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0register\t\r \u00C2\u00A0data\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0updated,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0beneficial\t\r \u00C2\u00A0to\t\r \u00C2\u00A0link\t\r \u00C2\u00A0the\t\r \u00C2\u00A0register\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0electronic\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0records\t\r \u00C2\u00A0(EMR)\t\r \u00C2\u00A0at\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic.\t\r \u00C2\u00A0\t\r \u00C2\u00A0This\t\r \u00C2\u00A0would\t\r \u00C2\u00A0allow\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0of\t\r \u00C2\u00A0new\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0information\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0automatically\t\r \u00C2\u00A0added\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 82\t\r \u00C2\u00A0 database.\t\r \u00C2\u00A0\t\r \u00C2\u00A0As\t\r \u00C2\u00A0only\t\r \u00C2\u00A0specific\t\r \u00C2\u00A0information\t\r \u00C2\u00A0will\t\r \u00C2\u00A0be\t\r \u00C2\u00A0required\t\r \u00C2\u00A0for\t\r \u00C2\u00A0transfer\t\r \u00C2\u00A0from\t\r \u00C2\u00A0EMR\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0register,\t\r \u00C2\u00A0specialized\t\r \u00C2\u00A0programming\t\r \u00C2\u00A0software\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0required\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0step.\t\r \u00C2\u00A0\t\r \u00C2\u00A0The\t\r \u00C2\u00A0alternative\t\r \u00C2\u00A0to\t\r \u00C2\u00A0this\t\r \u00C2\u00A0would\t\r \u00C2\u00A0involve\t\r \u00C2\u00A0an\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0responsible\t\r \u00C2\u00A0for\t\r \u00C2\u00A0data-\u00C2\u00AD\u00E2\u0080\u0090upkeep.\t\r \u00C2\u00A0\t\r \u00C2\u00A08)\t\r \u00C2\u00A0In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0ensure\t\r \u00C2\u00A0every\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0is\t\r \u00C2\u00A0living\t\r \u00C2\u00A0and\t\r \u00C2\u00A0in\t\r \u00C2\u00A0turn\t\r \u00C2\u00A0the\t\r \u00C2\u00A0register\t\r \u00C2\u00A0is\t\r \u00C2\u00A0constantly\t\r \u00C2\u00A0up\t\r \u00C2\u00A0to\t\r \u00C2\u00A0date,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0register\t\r \u00C2\u00A0should\t\r \u00C2\u00A0be\t\r \u00C2\u00A0linked\t\r \u00C2\u00A0to\t\r \u00C2\u00A0death\t\r \u00C2\u00A0records.\t\r \u00C2\u00A0\t\r \u00C2\u00A0An\t\r \u00C2\u00A0application\t\r \u00C2\u00A0to\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Population\t\r \u00C2\u00A0Data\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0must\t\r \u00C2\u00A0be\t\r \u00C2\u00A0made\t\r \u00C2\u00A0in\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0request\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0to\t\r \u00C2\u00A0death\t\r \u00C2\u00A0records\t\r \u00C2\u00A0(Population\t\r \u00C2\u00A0Data\t\r \u00C2\u00A0BC\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A09)\t\r \u00C2\u00A0Finally,\t\r \u00C2\u00A0as\t\r \u00C2\u00A0the\t\r \u00C2\u00A0database\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0designed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0mirror\t\r \u00C2\u00A0fields\t\r \u00C2\u00A0collected\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0network\t\r \u00C2\u00A0(EHDN)\t\r \u00C2\u00A0registry,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0register\t\r \u00C2\u00A0may\t\r \u00C2\u00A0be\t\r \u00C2\u00A0well\t\r \u00C2\u00A0suited\t\r \u00C2\u00A0for\t\r \u00C2\u00A0collaborating\t\r \u00C2\u00A0with\t\r \u00C2\u00A0this\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0potential\t\r \u00C2\u00A0to\t\r \u00C2\u00A0begin\t\r \u00C2\u00A0an\t\r \u00C2\u00A0international\t\r \u00C2\u00A0register\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Figure\t\r \u00C2\u00A027.\t\r \u00C2\u00A0Steps\t\r \u00C2\u00A0to\t\r \u00C2\u00A0be\t\r \u00C2\u00A0taken\t\r \u00C2\u00A0to\t\r \u00C2\u00A0convert\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0research\t\r \u00C2\u00A0database\t\r \u00C2\u00A0into\t\r \u00C2\u00A0a\t\r \u00C2\u00A0register\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 83\t\r \u00C2\u00A0 Bibliography\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Adachi\t\r \u00C2\u00A0Y,\t\r \u00C2\u00A0Nakashima\t\r \u00C2\u00A0K.\t\r \u00C2\u00A0\"Population\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0study\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease-\u00C2\u00AD\u00E2\u0080\u0090prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0founder's\t\r \u00C2\u00A0effect\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0San-\u00C2\u00AD\u00E2\u0080\u0090in\t\r \u00C2\u00A0area,\t\r \u00C2\u00A0western\t\r \u00C2\u00A0Japan.\"\t\r \u00C2\u00A0Nihon\t\r \u00C2\u00A0Rinsho\t\r \u00C2\u00A04\t\r \u00C2\u00A0(1999):\t\r \u00C2\u00A0100-\u00C2\u00AD\u00E2\u0080\u0090104.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader,\t\r \u00C2\u00A0LN,\t\r \u00C2\u00A0PS\t\r \u00C2\u00A0Harper,\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Krawczak,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0SR\t\r \u00C2\u00A0Palmer.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0frequency\t\r \u00C2\u00A0of\t\r \u00C2\u00A0inherited\t\r \u00C2\u00A0disorders\t\r \u00C2\u00A0database:\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Community\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02001:\t\r \u00C2\u00A0148-\u00C2\u00AD\u00E2\u0080\u0090157.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Almqvist\t\r \u00C2\u00A0EW,\t\r \u00C2\u00A0Elterman\t\r \u00C2\u00A0DS,\t\r \u00C2\u00A0MacLeod\t\r \u00C2\u00A0PM,\t\r \u00C2\u00A0Hayden\t\r \u00C2\u00A0MR.\t\r \u00C2\u00A0\"High\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0rate\t\r \u00C2\u00A0and\t\r \u00C2\u00A0absent\t\r \u00C2\u00A0family\t\r \u00C2\u00A0histories\t\r \u00C2\u00A0in\t\r \u00C2\u00A0one\t\r \u00C2\u00A0quarter\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0newly\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0 Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02001:\t\r \u00C2\u00A0198-\u00C2\u00AD\u00E2\u0080\u0090205.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Alonso\t\r \u00C2\u00A0,\t\r \u00C2\u00A0ME,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0\"Clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0characteristics\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Mexican\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0patients.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Movement\t\r \u00C2\u00A0Disorders,\t\r \u00C2\u00A02009:\t\r \u00C2\u00A02012-\u00C2\u00AD\u00E2\u0080\u00902015.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Avila-\u00C2\u00AD\u00E2\u0080\u0090Giron.\t\r \u00C2\u00A01973.\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Social\t\r \u00C2\u00A0Aspects\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0state\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Zulia,\t\r \u00C2\u00A0Venezuela.\t\r \u00C2\u00A0Advances\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Neurology\t\r \u00C2\u00A01:\t\r \u00C2\u00A0261\u00E2\u0080\u00936.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Barbeau,\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0C\t\r \u00C2\u00A0Coiteux,\t\r \u00C2\u00A0JG\t\r \u00C2\u00A0Trudeau,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Fullum\t\r \u00C2\u00A0G.\t\r \u00C2\u00A0\"Le\t\r \u00C2\u00A0Choree\t\r \u00C2\u00A0de\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Chez\t\r \u00C2\u00A0le\t\r \u00C2\u00A0Canadiens\t\r \u00C2\u00A0Francais:\t\r \u00C2\u00A0Etude\t\r \u00C2\u00A0Preliminaire.\"\t\r \u00C2\u00A0L'Union\t\r \u00C2\u00A0Medicale\t\r \u00C2\u00A0du\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A01964:\t\r \u00C2\u00A01178-\u00C2\u00AD\u00E2\u0080\u00901182.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Barbeau,\t\r \u00C2\u00A0Andre,\t\r \u00C2\u00A0Carl\t\r \u00C2\u00A0Coiteux,\t\r \u00C2\u00A0Jean-\u00C2\u00AD\u00E2\u0080\u0090Guy\t\r \u00C2\u00A0Trudeau,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Gabrielle\t\r \u00C2\u00A0Fullum.\t\r \u00C2\u00A0\"La\t\r \u00C2\u00A0Choree\t\r \u00C2\u00A0de\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0chez\t\r \u00C2\u00A0le\t\r \u00C2\u00A0Canadiens\t\r \u00C2\u00A0Francais\t\r \u00C2\u00A0Etude\t\r \u00C2\u00A0Preliminaire.\"\t\r \u00C2\u00A0L'Union\t\r \u00C2\u00A0Medicale\t\r \u00C2\u00A0du\t\r \u00C2\u00A0Canada,\t\r \u00C2\u00A01964:\t\r \u00C2\u00A01178-\u00C2\u00AD\u00E2\u0080\u00901182.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 BC,\t\r \u00C2\u00A0Stats.\t\r \u00C2\u00A0Population\t\r \u00C2\u00A0Projections.\t\r \u00C2\u00A001\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0http://www.bcstats.gov.bc.ca/StatisticsBySubject/Demography/PopulationProjections.aspx\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A003\t\r \u00C2\u00A027,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Bernhardt\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Anne-\u00C2\u00AD\u00E2\u0080\u0090Marie\t\r \u00C2\u00A0Schwan,\t\r \u00C2\u00A0Peter\t\r \u00C2\u00A0Kraus,\t\r \u00C2\u00A0Joerg\t\r \u00C2\u00A0Thomas\t\r \u00C2\u00A0Epplen\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Erdmute\t\r \u00C2\u00A0Kunstmann.\t\r \u00C2\u00A0\"Decreasing\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0German\t\r \u00C2\u00A0centre:\t\r \u00C2\u00A012\t\r \u00C2\u00A0years\u00E2\u0080\u0099\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0(1993\u00E2\u0080\u00932004)\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A017\t\r \u00C2\u00A0(2009):\t\r \u00C2\u00A0295\u00E2\u0080\u0093300.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Bickford,\t\r \u00C2\u00A0JA,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0RM\t\r \u00C2\u00A0Ellison.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0high\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Duchy\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Cornwall.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Mental\t\r \u00C2\u00A0Science,\t\r \u00C2\u00A01953:\t\r \u00C2\u00A0291-\u00C2\u00AD\u00E2\u0080\u0090294.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Bolt,\t\r \u00C2\u00A0JM.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0West\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Scotland.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A01970:\t\r \u00C2\u00A0259-\u00C2\u00AD\u00E2\u0080\u0090270.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Brewis,\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0DC\t\r \u00C2\u00A0Poskanzer,\t\r \u00C2\u00A0C\t\r \u00C2\u00A0Rolland,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0H\t\r \u00C2\u00A0Miller.\t\r \u00C2\u00A0\"Neurological\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0an\t\r \u00C2\u00A0English\t\r \u00C2\u00A0city.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0 Acta\t\r \u00C2\u00A0Neurologica\t\r \u00C2\u00A0Scandinavica,\t\r \u00C2\u00A01966:\t\r \u00C2\u00A01-\u00C2\u00AD\u00E2\u0080\u009089.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Agency.\t\r \u00C2\u00A0Selected\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Status\t\r \u00C2\u00A0Indicators\t\r \u00C2\u00A0.\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0report,\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Agency,\t\r \u00C2\u00A02000-\u00C2\u00AD\u00E2\u0080\u00902009.\t\r \u00C2\u00A0Brothers,\t\r \u00C2\u00A0CR.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0Chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Tasmania.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Sci,\t\r \u00C2\u00A01964:\t\r \u00C2\u00A0405-\u00C2\u00AD\u00E2\u0080\u009020.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Chen\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Y\t\r \u00C2\u00A0Lai.\t\r \u00C2\u00A0\"Nationwide\t\r \u00C2\u00A0Population-\u00C2\u00AD\u00E2\u0080\u0090Based\t\r \u00C2\u00A0Epidemiologic\t\r \u00C2\u00A0Study\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Taiwan.\"\t\r \u00C2\u00A0Neuroepidemiology,\t\r \u00C2\u00A02010:\t\r \u00C2\u00A0250\u00E2\u0080\u0093254.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Cameron,\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0GA\t\r \u00C2\u00A0Venters.\t\r \u00C2\u00A0\"Some\t\r \u00C2\u00A0problems\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0Chorea.\"\t\r \u00C2\u00A0Scottish\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0 Journal,\t\r \u00C2\u00A01967:\t\r \u00C2\u00A0152\u00E2\u0080\u0093156.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Caro,\t\r \u00C2\u00A0AJ.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0an\t\r \u00C2\u00A0area\t\r \u00C2\u00A0of\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Anglia.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0 Royal\t\r \u00C2\u00A0College\t\r \u00C2\u00A0of\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Practitioners,\t\r \u00C2\u00A01977:\t\r \u00C2\u00A041-\u00C2\u00AD\u00E2\u0080\u009045.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 84\t\r \u00C2\u00A0 Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.cmmt.ubc.ca/outreach/hd-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A002\t\r \u00C2\u00A002,\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Chang\t\r \u00C2\u00A0CM,\t\r \u00C2\u00A0Yu\t\r \u00C2\u00A0YL,\t\r \u00C2\u00A0Fong\t\r \u00C2\u00A0KY,\t\r \u00C2\u00A0Wong\t\r \u00C2\u00A0MT,\t\r \u00C2\u00A0Chan\t\r \u00C2\u00A0YW,\t\r \u00C2\u00A0Ng\t\r \u00C2\u00A0TH,\t\r \u00C2\u00A0Leung\t\r \u00C2\u00A0CM,\t\r \u00C2\u00A0Chan\t\r \u00C2\u00A0V.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Hong\t\r \u00C2\u00A0Kong\t\r \u00C2\u00A0Chinese:\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0picture.\"\t\r \u00C2\u00A0Clin\t\r \u00C2\u00A0Exp\t\r \u00C2\u00A0Neurol.,\t\r \u00C2\u00A01994:\t\r \u00C2\u00A043-\u00C2\u00AD\u00E2\u0080\u009051.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 CHDI.\t\r \u00C2\u00A0Cure\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Initiative\t\r \u00C2\u00A0Foundation.\t\r \u00C2\u00A0http://www.highqfoundation.org/\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A003\t\r \u00C2\u00A001,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Chen\t\r \u00C2\u00A0KM,\t\r \u00C2\u00A0Brody\t\r \u00C2\u00A0JA,\t\r \u00C2\u00A0Kurland\t\r \u00C2\u00A0LT.\t\r \u00C2\u00A0\"Patterns\t\r \u00C2\u00A0of\t\r \u00C2\u00A0neurologic\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0on\t\r \u00C2\u00A0guam.\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A06\t\r \u00C2\u00A0(1968):\t\r \u00C2\u00A0573-\u00C2\u00AD\u00E2\u0080\u00908.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 CIA\t\r \u00C2\u00A0World\t\r \u00C2\u00A0Factbook.\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0fertility\t\r \u00C2\u00A0rate.\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0https://www.cia.gov/library/publications/the-\u00C2\u00AD\u00E2\u0080\u0090world-\u00C2\u00AD\u00E2\u0080\u0090factbook/rankorder/2127rank.html\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A004\t\r \u00C2\u00A03,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Conneally,\t\r \u00C2\u00A0M.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0Disease:\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Epidemiology.\"\t\r \u00C2\u00A0American\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0 Genetics,\t\r \u00C2\u00A01984:\t\r \u00C2\u00A0506-\u00C2\u00AD\u00E2\u0080\u0090526.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Creighton\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Almqvist\t\r \u00C2\u00A0EW,\t\r \u00C2\u00A0MacGregor\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0Fernandez\t\r \u00C2\u00A0B,\t\r \u00C2\u00A0Hogg\t\r \u00C2\u00A0H,\t\r \u00C2\u00A0Beis\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Welch\t\r \u00C2\u00A0JP,\t\r \u00C2\u00A0Riddell\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Lokkesmoe\t\r \u00C2\u00A0R,\t\r \u00C2\u00A0Khalifa\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0MacKenzie\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Sajoo\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Farrell\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Robert\t\r \u00C2\u00A0F,\t\r \u00C2\u00A0Shugar\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Summers\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Meschino\t\r \u00C2\u00A0W,\t\r \u00C2\u00A0Allingham-\u00C2\u00AD\u00E2\u0080\u0090Hawkins\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0Chiu\t\r \u00C2\u00A0T,\t\r \u00C2\u00A0Hunter\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Allanson\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Hare\t\r \u00C2\u00A0H,\t\r \u00C2\u00A0Schween\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Collins\t\r \u00C2\u00A0L,\t\r \u00C2\u00A0Sanders\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Greenberg\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Cardwell\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Lemire\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0MacLeod\t\r \u00C2\u00A0P,\t\r \u00C2\u00A0Hayden\t\r \u00C2\u00A0MR.\t\r \u00C2\u00A0\"Predictive,\t\r \u00C2\u00A0pre-\u00C2\u00AD\u00E2\u0080\u0090natal\t\r \u00C2\u00A0and\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0from\t\r \u00C2\u00A01987\t\r \u00C2\u00A0to\t\r \u00C2\u00A02000.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0 Genetics,\t\r \u00C2\u00A02003:\t\r \u00C2\u00A0462\u00E2\u0080\u0093475.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 European\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Network.\t\r \u00C2\u00A0About\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0REGISTRY.\t\r \u00C2\u00A02010.\t\r \u00C2\u00A0http://www.euro-\u00C2\u00AD\u00E2\u0080\u0090hd.net/html/registry/about/description\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A004\t\r \u00C2\u00A030,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0Evergreen\t\r \u00C2\u00A0Hamlets\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Fleetwood.\t\r \u00C2\u00A0Evergreen\t\r \u00C2\u00A0Cottages.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.evergreen-\u00C2\u00AD\u00E2\u0080\u0090cottages.com/\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Falush\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0Almqvist\t\r \u00C2\u00A0EW,\t\r \u00C2\u00A0Brinkmann\t\r \u00C2\u00A0RR,\t\r \u00C2\u00A0Iwasa\t\r \u00C2\u00A0Y,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Hayden\t\r \u00C2\u00A0MR.\t\r \u00C2\u00A0\"Measurement\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Mutational\t\r \u00C2\u00A0Flow\t\r \u00C2\u00A0Implies\t\r \u00C2\u00A0Both\t\r \u00C2\u00A0a\t\r \u00C2\u00A0High\t\r \u00C2\u00A0New-\u00C2\u00AD\u00E2\u0080\u0090Mutation\t\r \u00C2\u00A0Rate\t\r \u00C2\u00A0for\t\r \u00C2\u00A0\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Substantial\t\r \u00C2\u00A0Underascertainment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Late-\u00C2\u00AD\u00E2\u0080\u0090Onset\t\r \u00C2\u00A0Cases.\"\t\r \u00C2\u00A0Am.\t\r \u00C2\u00A0J.\t\r \u00C2\u00A0Hum.\t\r \u00C2\u00A0Genet.,\t\r \u00C2\u00A02000:\t\r \u00C2\u00A0373-\u00C2\u00AD\u00E2\u0080\u0090385.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Falush,\t\r \u00C2\u00A0Daniel.\t\r \u00C2\u00A0\"Haplotype\t\r \u00C2\u00A0Background,\t\r \u00C2\u00A0Repeat\t\r \u00C2\u00A0Length\t\r \u00C2\u00A0Evolution,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0American\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A085\t\r \u00C2\u00A0(2009):\t\r \u00C2\u00A0939-\u00C2\u00AD\u00E2\u0080\u0090942.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Farrer\t\r \u00C2\u00A0LA,\t\r \u00C2\u00A0R\t\r \u00C2\u00A0H\t\r \u00C2\u00A0Myers,\t\r \u00C2\u00A0L\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Cupples,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0P\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Conneally.\t\r \u00C2\u00A0\"Considerations\t\r \u00C2\u00A0in\t\r \u00C2\u00A0using\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0as\t\r \u00C2\u00A0a\t\r \u00C2\u00A0presymptomatic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01987:\t\r \u00C2\u00A0577\u00E2\u0080\u0093588.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Folstein,\t\r \u00C2\u00A0Susan\t\r \u00C2\u00A0E.\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0a\t\r \u00C2\u00A0disorder\t\r \u00C2\u00A0of\t\r \u00C2\u00A0families.\t\r \u00C2\u00A0Baltimore:\t\r \u00C2\u00A0Johns\t\r \u00C2\u00A0Hopkins\t\r \u00C2\u00A0University\t\r \u00C2\u00A0Press,\t\r \u00C2\u00A01989.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Folstein,\t\r \u00C2\u00A0Susan,\t\r \u00C2\u00A0Gary\t\r \u00C2\u00A0Chase,\t\r \u00C2\u00A0Wendy\t\r \u00C2\u00A0Wahl,\t\r \u00C2\u00A0Anne\t\r \u00C2\u00A0McDonnell,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Marshal\t\r \u00C2\u00A0Folstein.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Maryland:\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Aspects\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Racial\t\r \u00C2\u00A0Variation\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A01987:\t\r \u00C2\u00A0168-\u00C2\u00AD\u00E2\u0080\u0090179.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Foroud\t\r \u00C2\u00A0T,\t\r \u00C2\u00A0Gray\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Ivashina\t\r \u00C2\u00A0J.\t\r \u00C2\u00A0\"Differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0duration\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0onset.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Neurosurg\t\r \u00C2\u00A0Psychiatry\t\r \u00C2\u00A066\t\r \u00C2\u00A0(1999):\t\r \u00C2\u00A052\u00E2\u0080\u009356.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Fox\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Bloch\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Fahy\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Hayden\t\r \u00C2\u00A0MR.\t\r \u00C2\u00A0\"Predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0I.\t\r \u00C2\u00A0Description\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0pilot\t\r \u00C2\u00A0project\t\r \u00C2\u00A0in\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia.\"\t\r \u00C2\u00A0American\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A032\t\r \u00C2\u00A0(1989):\t\r \u00C2\u00A0211-\u00C2\u00AD\u00E2\u0080\u0090216.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 85\t\r \u00C2\u00A0 Frontali\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Malaspina\t\r \u00C2\u00A0P,\t\r \u00C2\u00A0Rossi\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Jacopini\t\r \u00C2\u00A0AG,\t\r \u00C2\u00A0Vivona\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0Pergola\t\r \u00C2\u00A0MS,\t\r \u00C2\u00A0Palena\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Novelletto\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0\"Epidemiological\t\r \u00C2\u00A0and\t\r \u00C2\u00A0linkage\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0on\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Italy.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01990:\t\r \u00C2\u00A0165-\u00C2\u00AD\u00E2\u0080\u0090170.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Garc\u00C3\u00ADa\t\r \u00C2\u00A0Ruiz\t\r \u00C2\u00A0PJ,\t\r \u00C2\u00A0G\u00C3\u00B3mez-\u00C2\u00AD\u00E2\u0080\u0090Tortosa\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0del\t\r \u00C2\u00A0Barrio\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Ben\u00C3\u00ADtez\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Morales\t\r \u00C2\u00A0B,\t\r \u00C2\u00A0Vela\t\r \u00C2\u00A0L,\t\r \u00C2\u00A0Castro\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Requena\t\r \u00C2\u00A0I.\t\r \u00C2\u00A0\"Senile\t\r \u00C2\u00A0chorea:\t\r \u00C2\u00A0a\t\r \u00C2\u00A0multicenter\t\r \u00C2\u00A0prospective\t\r \u00C2\u00A0study.\"\t\r \u00C2\u00A0Acta\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Scand\t\r \u00C2\u00A03\t\r \u00C2\u00A0(1997):\t\r \u00C2\u00A0180-\u00C2\u00AD\u00E2\u0080\u0090183.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Garner,\t\r \u00C2\u00A0John\t\r \u00C2\u00A0Newton\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Sarah.\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Registers\t\r \u00C2\u00A0in\t\r \u00C2\u00A0England.\t\r \u00C2\u00A0Department\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Policy\t\r \u00C2\u00A0Research\t\r \u00C2\u00A0Programme,\t\r \u00C2\u00A0University\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0,\t\r \u00C2\u00A0Oxford:\t\r \u00C2\u00A0Institute\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Sciences,\t\r \u00C2\u00A02002.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Gassivaro\t\r \u00C2\u00A0Gallo,\t\r \u00C2\u00A0Buhagiar,\t\r \u00C2\u00A0Cuschieri\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Viviani.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0(HD)\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Malta:\t\r \u00C2\u00A0Epidemiology\t\r \u00C2\u00A0and\t\r \u00C2\u00A0origins.\"\t\r \u00C2\u00A0International\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Anthropology\t\r \u00C2\u00A014,\t\r \u00C2\u00A0no.\t\r \u00C2\u00A02-\u00C2\u00AD\u00E2\u0080\u00903\t\r \u00C2\u00A0(1999):\t\r \u00C2\u00A0577-\u00C2\u00AD\u00E2\u0080\u0090589.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Goizet\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Lesca\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0D\u00C3\u00BCrr\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0French\t\r \u00C2\u00A0Group\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Presymptomatic\t\r \u00C2\u00A0Testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Neurogenetic\t\r \u00C2\u00A0Disorders.\t\r \u00C2\u00A0\"Presymptomatic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0and\t\r \u00C2\u00A0autosomal\t\r \u00C2\u00A0dominant\t\r \u00C2\u00A0cerebellar\t\r \u00C2\u00A0ataxias.\"\t\r \u00C2\u00A0Neurology\t\r \u00C2\u00A059\t\r \u00C2\u00A0(2002):\t\r \u00C2\u00A01330-\u00C2\u00AD\u00E2\u0080\u00906.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 Govoni\t\r \u00C2\u00A0V,\t\r \u00C2\u00A0Pavoni\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Granieri\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0Carreras\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Malag\u00C3\u00B9\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Gandini\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0Del\t\r \u00C2\u00A0Senno\t\r \u00C2\u00A0L.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0province\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Ferrara\t\r \u00C2\u00A0from\t\r \u00C2\u00A01971\t\r \u00C2\u00A0to\t\r \u00C2\u00A01987.\t\r \u00C2\u00A0Descriptive\t\r \u00C2\u00A0study.\"\t\r \u00C2\u00A0Riv\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A06\t\r \u00C2\u00A0(1988):\t\r \u00C2\u00A0235-\u00C2\u00AD\u00E2\u0080\u0090240.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Gudmundsson,\t\r \u00C2\u00A0KR.\t\r \u00C2\u00A0\"Prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0occurrence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0some\t\r \u00C2\u00A0rare\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Iceland.\"\t\r \u00C2\u00A0Acta\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Scand\t\r \u00C2\u00A01\t\r \u00C2\u00A0(1969):\t\r \u00C2\u00A0114-\u00C2\u00AD\u00E2\u0080\u0090118.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Gusella,\t\r \u00C2\u00A0James.\t\r \u00C2\u00A0Test\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease.\t\r \u00C2\u00A0patent,\t\r \u00C2\u00A0The\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0Corporation,\t\r \u00C2\u00A0Boston:\t\r \u00C2\u00A0United\t\r \u00C2\u00A0States\t\r \u00C2\u00A0Patent,\t\r \u00C2\u00A01987.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Handley\t\r \u00C2\u00A0OJ,\t\r \u00C2\u00A0Marleen\t\r \u00C2\u00A0van\t\r \u00C2\u00A0Walsem,\t\r \u00C2\u00A0Peter\t\r \u00C2\u00A0Juni,\t\r \u00C2\u00A0Anne-\u00C2\u00AD\u00E2\u0080\u0090Catherine\t\r \u00C2\u00A0Bachoud-\u00C2\u00AD\u00E2\u0080\u0090Levi,\t\r \u00C2\u00A0Anna\t\r \u00C2\u00A0Rita\t\r \u00C2\u00A0Bentivoglio,\t\r \u00C2\u00A0Raphael\t\r \u00C2\u00A0M.\t\r \u00C2\u00A0Bonelli,\t\r \u00C2\u00A0Jean-\u00C2\u00AD\u00E2\u0080\u0090Marc\t\r \u00C2\u00A0Burgunder,\t\r \u00C2\u00A0Joaquim\t\r \u00C2\u00A0Ferreira,\t\r \u00C2\u00A0Arvid\t\r \u00C2\u00A0Heiberg,\t\r \u00C2\u00A0J\u00C3\u00B8rgen\t\r \u00C2\u00A0Nielsen,\t\r \u00C2\u00A0Markku\t\r \u00C2\u00A0P\u00C3\u00A4iv\u00C3\u00A4rinta,\t\r \u00C2\u00A0Sven\t\r \u00C2\u00A0P\u00C3\u00A5lhagen,\t\r \u00C2\u00A0Mar\u00C3\u00ADa\t\r \u00C2\u00A0Ramos-\u00C2\u00AD\u00E2\u0080\u0090Arroyo,\t\r \u00C2\u00A0Raymund\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0Roos,\t\r \u00C2\u00A0Sarah\t\r \u00C2\u00A0\t\r \u00C2\u00A0,\t\r \u00C2\u00A0Tereza\t\r \u00C2\u00A0Uhrova,\t\r \u00C2\u00A0Wim\t\r \u00C2\u00A0Vandenberghe,\t\r \u00C2\u00A0Christine\t\r \u00C2\u00A0Verellen-\u00C2\u00AD\u00E2\u0080\u0090Dumoulin,\t\r \u00C2\u00A0Jacek\t\r \u00C2\u00A0Zaremba,\t\r \u00C2\u00A0Bernhard\t\r \u00C2\u00A0G.\t\r \u00C2\u00A0Landwehrmeyer\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Registry\t\r \u00C2\u00A0investigators\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Network.\t\r \u00C2\u00A0\"Study\t\r \u00C2\u00A0Protocol\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Registry\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0version\t\r \u00C2\u00A02.0\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Network\t\r \u00C2\u00A0(EHDN)\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Hygeia\t\r \u00C2\u00A0 Public\t\r \u00C2\u00A0Health,\t\r \u00C2\u00A02011:\t\r \u00C2\u00A0115-\u00C2\u00AD\u00E2\u0080\u0090182.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0PS,\t\r \u00C2\u00A0Lim\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Craufurd\t\r \u00C2\u00A0D.\t\r \u00C2\u00A0\"Ten\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0presymptomatic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Prediction\t\r \u00C2\u00A0Consortium.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0 Genetics,\t\r \u00C2\u00A02000:\t\r \u00C2\u00A0567\u00E2\u0080\u0093571.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Harper,\t\r \u00C2\u00A0Peter\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Newcombe,\t\r \u00C2\u00A0Robert.\t\r \u00C2\u00A0\"Age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0and\t\r \u00C2\u00A0life\t\r \u00C2\u00A0table\t\r \u00C2\u00A0risks\t\r \u00C2\u00A0in\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0counselling\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A029\t\r \u00C2\u00A0(1992):\t\r \u00C2\u00A0239-\u00C2\u00AD\u00E2\u0080\u0090242.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Harper,\t\r \u00C2\u00A0PS.\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0Disease.\t\r \u00C2\u00A0Cardiff:\t\r \u00C2\u00A0W.B.\t\r \u00C2\u00A0Saunders\t\r \u00C2\u00A0Company,\t\r \u00C2\u00A01996.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Harper,\t\r \u00C2\u00A0PS.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A089(1992):\t\r \u00C2\u00A0365-\u00C2\u00AD\u00E2\u0080\u0090376.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Hayden\t\r \u00C2\u00A0MR,\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A0P.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Cape\t\r \u00C2\u00A0coloured\t\r \u00C2\u00A0community\t\r \u00C2\u00A0of\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa.\"\t\r \u00C2\u00A0S\t\r \u00C2\u00A0Afr\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0J\t\r \u00C2\u00A022\t\r \u00C2\u00A0(1977):\t\r \u00C2\u00A0886-\u00C2\u00AD\u00E2\u0080\u0090888.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Hayden\t\r \u00C2\u00A0MR,\t\r \u00C2\u00A0Berkowicz\t\r \u00C2\u00A0AL,\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A0PH,\t\r \u00C2\u00A0Yiptong\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0island\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Mauritius.\"\t\r \u00C2\u00A0South\t\r \u00C2\u00A0African\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A026\t\r \u00C2\u00A0(1981):\t\r \u00C2\u00A01001-\u00C2\u00AD\u00E2\u0080\u00902.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Hayden,\t\r \u00C2\u00A0MacGregor\t\r \u00C2\u00A0JM,\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A0PH.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa.\"\t\r \u00C2\u00A0S\t\r \u00C2\u00A0Afr\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0J\t\r \u00C2\u00A05\t\r \u00C2\u00A0(1980):\t\r \u00C2\u00A0193-\u00C2\u00AD\u00E2\u0080\u0090196.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 86\t\r \u00C2\u00A0 He\u00C4\u0087imovi\u00C4\u0087\t\r \u00C2\u00A0,\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0\"Genetic\t\r \u00C2\u00A0background\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Croatia:\t\r \u00C2\u00A0Molecular\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0CAG,\t\r \u00C2\u00A0CCG,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Delta2642\t\r \u00C2\u00A0(E2642del)\t\r \u00C2\u00A0polymorphisms.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Mutation,\t\r \u00C2\u00A02002:\t\r \u00C2\u00A0233.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Heemskerk\t\r \u00C2\u00A0AW,\t\r \u00C2\u00A0R\t\r \u00C2\u00A0A\t\r \u00C2\u00A0C\t\r \u00C2\u00A0Roos.\t\r \u00C2\u00A0\"E04\t\r \u00C2\u00A0Causes\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0 Neurosurg\t\r \u00C2\u00A0Psychiatry\t\r \u00C2\u00A081\t\r \u00C2\u00A0(2010):\t\r \u00C2\u00A0A22.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Heathfield,\t\r \u00C2\u00A0KW.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea.\t\r \u00C2\u00A0Investigation\t\r \u00C2\u00A0into\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0this\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0area\t\r \u00C2\u00A0covered\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0North\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Metropolitan\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0Board.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Brain:\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0 Neurology,\t\r \u00C2\u00A01967:\t\r \u00C2\u00A0203-\u00C2\u00AD\u00E2\u0080\u0090232.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Huntington's\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Collaborative\t\r \u00C2\u00A0Research\t\r \u00C2\u00A0Group.\t\r \u00C2\u00A0\"\u0000A\t\r \u00C2\u00A0Novel\t\r \u00C2\u00A0Gene\t\r \u00C2\u00A0Containing\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Trinucleotide\t\r \u00C2\u00A0Repeat\t\r \u00C2\u00A0That\t\r \u00C2\u00A0Is\t\r \u00C2\u00A0Expanded\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Unstable\t\r \u00C2\u00A0on\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Chromosomes\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Cell,\t\r \u00C2\u00A01993:\t\r \u00C2\u00A0971-\u00C2\u00AD\u00E2\u0080\u0090983.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Huntington\t\r \u00C2\u00A0G.\t\r \u00C2\u00A0\"On\t\r \u00C2\u00A0Chorea\".\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Surgical\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Philadelphia.,\t\r \u00C2\u00A026(15):\t\r \u00C2\u00A0317-\u00C2\u00AD\u00E2\u0080\u0090321.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Huntington\t\r \u00C2\u00A0Study\t\r \u00C2\u00A0Group.\t\r \u00C2\u00A0\"Unified\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Rating\t\r \u00C2\u00A0Scale:\t\r \u00C2\u00A0Reliability\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Consistency\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Movement\t\r \u00C2\u00A0Disorders\t\r \u00C2\u00A011,\t\r \u00C2\u00A0no.\t\r \u00C2\u00A02\t\r \u00C2\u00A0(1996):\t\r \u00C2\u00A0136-\u00C2\u00AD\u00E2\u0080\u0090142.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Husquinet,\t\r \u00C2\u00A0H.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0social\t\r \u00C2\u00A0problem;\t\r \u00C2\u00A0eugenic\t\r \u00C2\u00A0problem.\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Belg\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0Soc.,\t\r \u00C2\u00A01972:\t\r \u00C2\u00A065-\u00C2\u00AD\u00E2\u0080\u009074.\t\r \u00C2\u00A0InternationalEnergyAgency.\t\r \u00C2\u00A0Key\t\r \u00C2\u00A0World\t\r \u00C2\u00A0Energy\t\r \u00C2\u00A0Statistics.\t\r \u00C2\u00A0Paris:\t\r \u00C2\u00A0IEA,\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Kandil\t\r \u00C2\u00A0MR,\t\r \u00C2\u00A0Tohamy\t\r \u00C2\u00A0SA,\t\r \u00C2\u00A0Fattah\t\r \u00C2\u00A0MA,\t\r \u00C2\u00A0Ahmed\t\r \u00C2\u00A0HN,\t\r \u00C2\u00A0Farwiez\t\r \u00C2\u00A0HM.\t\r \u00C2\u00A0\"Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0chorea,\t\r \u00C2\u00A0dystonia\t\r \u00C2\u00A0and\t\r \u00C2\u00A0athetosis\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Assiut,\t\r \u00C2\u00A0Egypt:\t\r \u00C2\u00A0a\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0study.\"\t\r \u00C2\u00A0Neuroepidemiology\t\r \u00C2\u00A05\t\r \u00C2\u00A0(1994):\t\r \u00C2\u00A0202-\u00C2\u00AD\u00E2\u0080\u0090210.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Kirilenko\t\r \u00C2\u00A0NB,\t\r \u00C2\u00A0Fedotov\t\r \u00C2\u00A0VP,\t\r \u00C2\u00A0Baryshnikova\t\r \u00C2\u00A0NV,\t\r \u00C2\u00A0Dadali\t\r \u00C2\u00A0EL,\t\r \u00C2\u00A0Poliakov\t\r \u00C2\u00A0AV.\t\r \u00C2\u00A0\"Nozological\t\r \u00C2\u00A0spectrum\t\r \u00C2\u00A0of\t\r \u00C2\u00A0hereditary\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0nervous\t\r \u00C2\u00A0system\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Volgograd\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Volzhsky.\"\t\r \u00C2\u00A0Genetika\t\r \u00C2\u00A09\t\r \u00C2\u00A0(2004):\t\r \u00C2\u00A01262-\u00C2\u00AD\u00E2\u0080\u00907.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Kirkwood,\t\r \u00C2\u00A0Sandra,\t\r \u00C2\u00A0Jessica\t\r \u00C2\u00A0Su,\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0Conneally,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Tatiana\t\r \u00C2\u00A0Foroud.\t\r \u00C2\u00A0\"Progression\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Symptoms\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Early\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Middle\t\r \u00C2\u00A0Stages\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Neurol,\t\r \u00C2\u00A02001:\t\r \u00C2\u00A0273-\u00C2\u00AD\u00E2\u0080\u0090278.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Kokmen\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0Ozekmek\u00C3\u00A7i\t\r \u00C2\u00A0FS,\t\r \u00C2\u00A0Beard\t\r \u00C2\u00A0CM,\t\r \u00C2\u00A0O'Brien\t\r \u00C2\u00A0PC,\t\r \u00C2\u00A0Kurland\t\r \u00C2\u00A0LT.\t\r \u00C2\u00A0\"Incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Olmsted\t\r \u00C2\u00A0County,\t\r \u00C2\u00A0Minnesota\t\r \u00C2\u00A0(1950\t\r \u00C2\u00A0Through\t\r \u00C2\u00A01989).\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Neurol,\t\r \u00C2\u00A01994:\t\r \u00C2\u00A0696-\u00C2\u00AD\u00E2\u0080\u0090698.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Laccone\t\r \u00C2\u00A0F,\t\r \u00C2\u00A0Engel\t\r \u00C2\u00A0U,\t\r \u00C2\u00A0Holinski-\u00C2\u00AD\u00E2\u0080\u0090Feder\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0Weigell-\u00C2\u00AD\u00E2\u0080\u0090Weber\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Marczinek\t\r \u00C2\u00A0K,\t\r \u00C2\u00A0Nolte\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0Morris-\u00C2\u00AD\u00E2\u0080\u0090Rosendahl\t\r \u00C2\u00A0DJ,\t\r \u00C2\u00A0Z\u00C3\u00BChlke\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Fuchs\t\r \u00C2\u00A0K,\t\r \u00C2\u00A0Weirich-\u00C2\u00AD\u00E2\u0080\u0090Schwaiger\t\r \u00C2\u00A0H,\t\r \u00C2\u00A0Schl\u00C3\u00BCter\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0von\t\r \u00C2\u00A0Beust\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0Vieira-\u00C2\u00AD\u00E2\u0080\u0090Saecker\t\r \u00C2\u00A0AM,\t\r \u00C2\u00A0Weber\t\r \u00C2\u00A0BH,\t\r \u00C2\u00A0Riess\t\r \u00C2\u00A0O.\t\r \u00C2\u00A0\"DNA\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0five\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Germany,\t\r \u00C2\u00A0Austria,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Switzerland.\"\t\r \u00C2\u00A0Neurology\t\r \u00C2\u00A04\t\r \u00C2\u00A0(1999):\t\r \u00C2\u00A0801-\u00C2\u00AD\u00E2\u0080\u0090806.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Langbehn,\t\r \u00C2\u00A0DR,\t\r \u00C2\u00A0RR\t\r \u00C2\u00A0Brinkman,\t\r \u00C2\u00A0D\t\r \u00C2\u00A0Falush,\t\r \u00C2\u00A0JS\t\r \u00C2\u00A0Paulsen,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0MR\t\r \u00C2\u00A0Hayden.\t\r \u00C2\u00A0\"A\t\r \u00C2\u00A0new\t\r \u00C2\u00A0model\t\r \u00C2\u00A0for\t\r \u00C2\u00A0prediction\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0and\t\r \u00C2\u00A0penetrance\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0based\t\r \u00C2\u00A0on\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0length\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0 Genetics,\t\r \u00C2\u00A02004:\t\r \u00C2\u00A0267\u00E2\u0080\u0093277.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Levy,\t\r \u00C2\u00A0Micheline\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Fenigold,\t\r \u00C2\u00A0Josue.\t\r \u00C2\u00A0\"Estimating\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0in\t\r \u00C2\u00A0single-\u00C2\u00AD\u00E2\u0080\u0090gene\t\r \u00C2\u00A0kidney\t\r \u00C2\u00A0diseases\t\r \u00C2\u00A0progressing\t\r \u00C2\u00A0to\t\r \u00C2\u00A0renal\t\r \u00C2\u00A0failure.\"\t\r \u00C2\u00A0Kidney\t\r \u00C2\u00A0International\t\r \u00C2\u00A058\t\r \u00C2\u00A0(2000):\t\r \u00C2\u00A0925\u00E2\u0080\u0093943.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Long\t\r \u00C2\u00A0term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0Canada.\t\r \u00C2\u00A0Nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0living\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0know\t\r \u00C2\u00A0the\t\r \u00C2\u00A0facts.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.longtermcarecanada.com/long_term_care_resources/care_years_pg_9.html\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 87\t\r \u00C2\u00A0 Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Vegter-\u00C2\u00AD\u00E2\u0080\u0090van\t\r \u00C2\u00A0der\t\r \u00C2\u00A0Vlis\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Zoeteweij\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Losekoot\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0van\t\r \u00C2\u00A0Haeringen\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Roos\t\r \u00C2\u00A0R.\t\r \u00C2\u00A0\"is\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Zoeteweij\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Losekoot\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0van\t\r \u00C2\u00A0Haeringen\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Roos\t\r \u00C2\u00A0R:\t\r \u00C2\u00A0Paradox\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0better\t\r \u00C2\u00A0test\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Neurosurg\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A02000:\t\r \u00C2\u00A0579\u00E2\u0080\u0093583.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Macdonald\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Christine\t\r \u00C2\u00A0M.\t\r \u00C2\u00A0Ambrose,\t\r \u00C2\u00A0Mabel\t\r \u00C2\u00A0P.\t\r \u00C2\u00A0Duyao,\t\r \u00C2\u00A0Richard\t\r \u00C2\u00A0H.\t\r \u00C2\u00A0Myers,\t\r \u00C2\u00A0Carol\t\r \u00C2\u00A0Lin,\t\r \u00C2\u00A0Lakshmi\t\r \u00C2\u00A0Srinidhi,\t\r \u00C2\u00A0Glenn\t\r \u00C2\u00A0Barnes,\t\r \u00C2\u00A0Sherry\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0Taylor,\t\r \u00C2\u00A0Marianne\t\r \u00C2\u00A0James,\t\r \u00C2\u00A0Nicolet\t\r \u00C2\u00A0Groat,\t\r \u00C2\u00A0Heather\t\r \u00C2\u00A0MacFarlane,\t\r \u00C2\u00A0Barbara\t\r \u00C2\u00A0Jenkins,\t\r \u00C2\u00A0Mary\t\r \u00C2\u00A0Anne\t\r \u00C2\u00A0Anderson,\t\r \u00C2\u00A0Nancy\t\r \u00C2\u00A0S.\t\r \u00C2\u00A0Wexler\t\r \u00C2\u00A0and\t\r \u00C2\u00A0James\t\r \u00C2\u00A0F.\t\r \u00C2\u00A0Gusella.\t\r \u00C2\u00A0\u00E2\u0080\u009CA\t\r \u00C2\u00A0Novel\t\r \u00C2\u00A0Gene\t\r \u00C2\u00A0Containing\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Trinucleotide\t\r \u00C2\u00A0Repeat\t\r \u00C2\u00A0That\t\r \u00C2\u00A0Is\t\r \u00C2\u00A0Expanded\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Unstable\t\r \u00C2\u00A0on\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Chromosomes\u00E2\u0080\u009D.\t\r \u00C2\u00A0Cell.\t\r \u00C2\u00A01993.\t\r \u00C2\u00A072:971-\u00C2\u00AD\u00E2\u0080\u0090983\t\r \u00C2\u00A0MacLeod\t\r \u00C2\u00A0R,\t\r \u00C2\u00A0Tibben\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Frontali\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Evers\t\r \u00C2\u00A0Kiebooms\t\r \u00C2\u00A0G\t\r \u00C2\u00A0Jones\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Martinez\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0writing\t\r \u00C2\u00A0committee\t\r \u00C2\u00A0and\t\r \u00C2\u00A0working\t\r \u00C2\u00A0group\t\r \u00C2\u00A0\u00E2\u0080\u0098Genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Counselling\u00E2\u0080\u0099\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0EHDN.\t\r \u00C2\u00A0RECOMMENDATIONS\t\r \u00C2\u00A0 FOR\t\r \u00C2\u00A0THE\t\r \u00C2\u00A0MOLECULAR\t\r \u00C2\u00A0GENETICS\t\r \u00C2\u00A0PREDICTIVE\t\r \u00C2\u00A0TEST\t\r \u00C2\u00A0IN\t\r \u00C2\u00A0HUNTINGTON\t\r \u00C2\u00A0DISEASE\t\r \u00C2\u00A0.\t\r \u00C2\u00A0Guidelines,\t\r \u00C2\u00A0Writing\t\r \u00C2\u00A0committee:\t\r \u00C2\u00A0Martin\t\r \u00C2\u00A0Delatycki,\t\r \u00C2\u00A0Mark\t\r \u00C2\u00A0Guttman,\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0Hayden,\t\r \u00C2\u00A0Ann\t\r \u00C2\u00A0Jones,\t\r \u00C2\u00A0Rhona\t\r \u00C2\u00A0MacLeod,\t\r \u00C2\u00A0Asunci\u00C3\u00B3n\t\r \u00C2\u00A0Mart\u00C3\u00ADnez\t\r \u00C2\u00A0Descales,\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 MacMillan\t\r \u00C2\u00A0JC,\t\r \u00C2\u00A0Snell\t\r \u00C2\u00A0RG,\t\r \u00C2\u00A0Tyler\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Houlihan\t\r \u00C2\u00A0GD,\t\r \u00C2\u00A0Fenton\t\r \u00C2\u00A0SRN,\t\r \u00C2\u00A0Cheadle\t\r \u00C2\u00A0JP,\t\r \u00C2\u00A0Lazarou\t\r \u00C2\u00A0LP,\t\r \u00C2\u00A0Shaw\t\r \u00C2\u00A0JD\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A0PS.\t\r \u00C2\u00A0\"Molecular\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0correlations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0mutation\".\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0Lancet,\t\r \u00C2\u00A01993.\t\r \u00C2\u00A0342(8877)954-\u00C2\u00AD\u00E2\u0080\u0090958.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 MacMillan,\t\r \u00C2\u00A0JC,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0PS\t\r \u00C2\u00A0Harper.\t\r \u00C2\u00A0\"Single-\u00C2\u00AD\u00E2\u0080\u0090gene\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0disorders\t\r \u00C2\u00A0in\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales:\t\r \u00C2\u00A0an\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0study\".\t\r \u00C2\u00A0Annals\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Neurology,\t\r \u00C2\u00A01991:\t\r \u00C2\u00A0411-\u00C2\u00AD\u00E2\u0080\u0090414.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Massey\t\r \u00C2\u00A0University.\t\r \u00C2\u00A0Estimate\t\r \u00C2\u00A0prevalence:\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0interval.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.promesa.co.nz/Help/EP_est_simple_random_sample.htm\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A020\t\r \u00C2\u00A005,\t\r \u00C2\u00A02012)\t\r \u00C2\u00A0\t\r \u00C2\u00A0 McCusker\t\r \u00C2\u00A0EA,\t\r \u00C2\u00A0Casse\t\r \u00C2\u00A0RF,\t\r \u00C2\u00A0Graham\t\r \u00C2\u00A0SJ,\t\r \u00C2\u00A0Williams\t\r \u00C2\u00A0DB,\t\r \u00C2\u00A0Lazarus\t\r \u00C2\u00A0R.\t\r \u00C2\u00A0\"Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0New\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0in\t\r \u00C2\u00A01996.\"\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Aust\t\r \u00C2\u00A04\t\r \u00C2\u00A0(2000):\t\r \u00C2\u00A0187-\u00C2\u00AD\u00E2\u0080\u0090190.\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0News.\t\r \u00C2\u00A0Rising\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0dementia\t\r \u00C2\u00A0will\t\r \u00C2\u00A0cripple\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0families,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0health\t\r \u00C2\u00A0care\t\r \u00C2\u00A0system\t\r \u00C2\u00A0and\t\r \u00C2\u00A0 economy.\t\r \u00C2\u00A0Jan\t\r \u00C2\u00A04,\t\r \u00C2\u00A02010.\t\r \u00C2\u00A0http://www.news-\u00C2\u00AD\u00E2\u0080\u0090medical.net/news/20100104/Rising-\u00C2\u00AD\u00E2\u0080\u0090prevalence-\u00C2\u00AD\u00E2\u0080\u0090of-\u00C2\u00AD\u00E2\u0080\u0090dementia-\u00C2\u00AD\u00E2\u0080\u0090will-\u00C2\u00AD\u00E2\u0080\u0090cripple-\u00C2\u00AD\u00E2\u0080\u0090Canadian-\u00C2\u00AD\u00E2\u0080\u0090families-\u00C2\u00AD\u00E2\u0080\u0090the-\u00C2\u00AD\u00E2\u0080\u0090health-\u00C2\u00AD\u00E2\u0080\u0090care-\u00C2\u00AD\u00E2\u0080\u0090system-\u00C2\u00AD\u00E2\u0080\u0090and-\u00C2\u00AD\u00E2\u0080\u0090economy.aspx\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A0April\t\r \u00C2\u00A029,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Ministry\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Health.\t\r \u00C2\u00A0eHealth\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0Faster,\t\r \u00C2\u00A0safer,\t\r \u00C2\u00A0better\t\r \u00C2\u00A0healthcare.\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0http://www.health.gov.bc.ca/ehealth/telehealth.html\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A005\t\r \u00C2\u00A008,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0Morrison.\t\r \u00C2\u00A0\"Morrison\t\r \u00C2\u00A0PJ,\t\r \u00C2\u00A0Harding-\u00C2\u00AD\u00E2\u0080\u0090Lester\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Bradley\t\r \u00C2\u00A0A.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A080\t\r \u00C2\u00A0(2011):\t\r \u00C2\u00A0281\u00E2\u0080\u0093286.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Morrison,\t\r \u00C2\u00A0PJ,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0NC\t\r \u00C2\u00A0Nevin.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0County\t\r \u00C2\u00A0Donegal:\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0trends\t\r \u00C2\u00A0over\t\r \u00C2\u00A0four\t\r \u00C2\u00A0decades.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Ulster\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Journal,\t\r \u00C2\u00A01993:\t\r \u00C2\u00A0141-\u00C2\u00AD\u00E2\u0080\u0090144.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Morrison,\t\r \u00C2\u00A0PJ,\t\r \u00C2\u00A0WP\t\r \u00C2\u00A0Johnston,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0NC\t\r \u00C2\u00A0Nevin.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01995:\t\r \u00C2\u00A0524-\u00C2\u00AD\u00E2\u0080\u0090530.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Morrison,\t\r \u00C2\u00A0PJ,\t\r \u00C2\u00A0Harding-\u00C2\u00AD\u00E2\u0080\u0090Lester\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Bradley\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0\"Uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0a\t\r \u00C2\u00A0complete\t\r \u00C2\u00A0population.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02010:\t\r \u00C2\u00A0281-\u00C2\u00AD\u00E2\u0080\u0090286.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Myrianthopoulos.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0Chorea.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0Genet,\t\r \u00C2\u00A01966:\t\r \u00C2\u00A0298-\u00C2\u00AD\u00E2\u0080\u0090314.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Nance,\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Fiona\t\r \u00C2\u00A0Richards,\t\r \u00C2\u00A0Raymund\t\r \u00C2\u00A0Roos,\t\r \u00C2\u00A0Aad\t\r \u00C2\u00A0Tibben,\t\r \u00C2\u00A0Louise\t\r \u00C2\u00A0Vetter,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Oliver,\t\r \u00C2\u00A0JE.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Northamptonshire.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A01970:\t\r \u00C2\u00A0241-\u00C2\u00AD\u00E2\u0080\u0090253.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 88\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Orth\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Schwenke\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0\"Age-\u00C2\u00AD\u00E2\u0080\u0090at-\u00C2\u00AD\u00E2\u0080\u0090onset\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0PLoS\t\r \u00C2\u00A0Currents\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0 Disease,\t\r \u00C2\u00A02011:\t\r \u00C2\u00A0doi:\t\r \u00C2\u00A010.1371.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Panas\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Karadima\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0Vassos\t\r \u00C2\u00A0E,\t\r \u00C2\u00A0Kalfakis\t\r \u00C2\u00A0N,\t\r \u00C2\u00A0Kladi\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Christodoulou\t\r \u00C2\u00A0K,\t\r \u00C2\u00A0Vassilopoulos\t\r \u00C2\u00A0D.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Greece:\t\r \u00C2\u00A0the\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0of\t\r \u00C2\u00A014\t\r \u00C2\u00A0years.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02011:\t\r \u00C2\u00A0586-\u00C2\u00AD\u00E2\u0080\u0090590.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Paradisi,\t\r \u00C2\u00A0I,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Hern\u00C3\u00A1ndez,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0S\t\r \u00C2\u00A0Arias.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Venezuela:\t\r \u00C2\u00A0age\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset,\t\r \u00C2\u00A0haplotype\t\r \u00C2\u00A0analyses\t\r \u00C2\u00A0and\t\r \u00C2\u00A0geographic\t\r \u00C2\u00A0aggregation.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02008:\t\r \u00C2\u00A0127-\u00C2\u00AD\u00E2\u0080\u0090135.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Peterlin,\t\r \u00C2\u00A0Kobal\t\r \u00C2\u00A0J,\t\r \u00C2\u00A0Teran\t\r \u00C2\u00A0N,\t\r \u00C2\u00A0Flisar\t\r \u00C2\u00A0D,\t\r \u00C2\u00A0Lovreci\u00C4\u0087\t\r \u00C2\u00A0L.\t\r \u00C2\u00A0\"Epidemiology\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Slovenia.\"\t\r \u00C2\u00A0Acta\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Scand\t\r \u00C2\u00A06\t\r \u00C2\u00A0(2009):\t\r \u00C2\u00A0371-\u00C2\u00AD\u00E2\u0080\u0090375.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Petit,\t\r \u00C2\u00A0H,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0JL\t\r \u00C2\u00A0Salomez.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease.\t\r \u00C2\u00A0Contribution\t\r \u00C2\u00A0of\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0data\t\r \u00C2\u00A0to\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0counseling.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0de\t\r \u00C2\u00A0Genetique\t\r \u00C2\u00A0Humaine,\t\r \u00C2\u00A01985:\t\r \u00C2\u00A091-\u00C2\u00AD\u00E2\u0080\u0090102.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Population\t\r \u00C2\u00A0Data\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0DATA\t\r \u00C2\u00A0SET:\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0VITAL\t\r \u00C2\u00A0STATISTICS\t\r \u00C2\u00A0DEATHS.\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0http://www.popdata.bc.ca/data/internal/demographic/vsdeaths\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A005\t\r \u00C2\u00A005,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Pridmore\t\r \u00C2\u00A0SA.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Tasmania.\"\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Aust\t\r \u00C2\u00A03\t\r \u00C2\u00A0(1990):\t\r \u00C2\u00A0133-\u00C2\u00AD\u00E2\u0080\u0090134.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Quarrell\t\r \u00C2\u00A0O,\t\r \u00C2\u00A0Alan\t\r \u00C2\u00A0Rigby,\t\r \u00C2\u00A0L\t\r \u00C2\u00A0Barron,\t\r \u00C2\u00A0Y\t\r \u00C2\u00A0Crow,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Dalton,\t\r \u00C2\u00A0N\t\r \u00C2\u00A0Dennis,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0E\t\r \u00C2\u00A0Fryer,\t\r \u00C2\u00A0F\t\r \u00C2\u00A0Heydon,\t\r \u00C2\u00A0E\t\r \u00C2\u00A0Kinning,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Lashwood,\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Losekoot,\t\r \u00C2\u00A0L\t\r \u00C2\u00A0Margerison,\t\r \u00C2\u00A0S\t\r \u00C2\u00A0McDonnell,\t\r \u00C2\u00A0P\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Morrison,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Norman,\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Peterson,\t\r \u00C2\u00A0F\t\r \u00C2\u00A0L\t\r \u00C2\u00A0Raymond,\t\r \u00C2\u00A0S\t\r \u00C2\u00A0Simpson,\t\r \u00C2\u00A0E\t\r \u00C2\u00A0Thompson,\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Warner.\t\r \u00C2\u00A0\"Reduced\t\r \u00C2\u00A0penetrance\t\r \u00C2\u00A0alleles\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease:\t\r \u00C2\u00A0a\t\r \u00C2\u00A0multi-\u00C2\u00AD\u00E2\u0080\u0090centre\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0observational\t\r \u00C2\u00A0study.\"\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A044,\t\r \u00C2\u00A0no.\t\r \u00C2\u00A03\t\r \u00C2\u00A0(2007):\t\r \u00C2\u00A0e68.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Quarrell\t\r \u00C2\u00A0,\t\r \u00C2\u00A0OW,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Tyler,\t\r \u00C2\u00A0MP\t\r \u00C2\u00A0Jones,\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Nordin,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0PS\t\r \u00C2\u00A0Harper.\t\r \u00C2\u00A0\"Population\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Wales.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01988:\t\r \u00C2\u00A0189-\u00C2\u00AD\u00E2\u0080\u0090195.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Ramos-\u00C2\u00AD\u00E2\u0080\u0090Arroyo\t\r \u00C2\u00A0MA,\t\r \u00C2\u00A0Moreno\t\r \u00C2\u00A0S,\t\r \u00C2\u00A0Valiente\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0\"Incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0rates\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Spain:\t\r \u00C2\u00A0experience\t\r \u00C2\u00A0of\t\r \u00C2\u00A09\t\r \u00C2\u00A0years\t\r \u00C2\u00A0of\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0testing.\"\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A0Neurosurg\t\r \u00C2\u00A0Psychiatry\t\r \u00C2\u00A03\t\r \u00C2\u00A0(2005):\t\r \u00C2\u00A0337-\u00C2\u00AD\u00E2\u0080\u0090342.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Rawlins,\t\r \u00C2\u00A0M.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0closet?\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Lancet\t\r \u00C2\u00A0Neurology,\t\r \u00C2\u00A02010:\t\r \u00C2\u00A01372-\u00C2\u00AD\u00E2\u0080\u00901373.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Reed,\t\r \u00C2\u00A0Edward,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Joseph\t\r \u00C2\u00A0Chandler.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0Chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Michigan:\t\r \u00C2\u00A0Demography\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Genetics.\"\t\r \u00C2\u00A01958:\t\r \u00C2\u00A0201-\u00C2\u00AD\u00E2\u0080\u0090225.\t\r \u00C2\u00A0Roccatagliata\t\r \u00C2\u00A0G,\t\r \u00C2\u00A0De\t\r \u00C2\u00A0Marchi\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0Maffini\t\r \u00C2\u00A0M,\t\r \u00C2\u00A0Albano\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0\"Epidemiological\t\r \u00C2\u00A0aspects\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Genoa\t\r \u00C2\u00A0region\t\r \u00C2\u00A0from\t\r \u00C2\u00A01930\t\r \u00C2\u00A0to\t\r \u00C2\u00A01977.\"\t\r \u00C2\u00A0Rivista\t\r \u00C2\u00A0di\t\r \u00C2\u00A0patologia\t\r \u00C2\u00A0nervosa\t\r \u00C2\u00A0e\t\r \u00C2\u00A0mentale\t\r \u00C2\u00A04\t\r \u00C2\u00A0(1983):\t\r \u00C2\u00A0171-\u00C2\u00AD\u00E2\u0080\u00907.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Rothman,\t\r \u00C2\u00A0Kenneth\t\r \u00C2\u00A0J.\t\r \u00C2\u00A0Epidemiology:\t\r \u00C2\u00A0an\t\r \u00C2\u00A0introduction.\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0University\t\r \u00C2\u00A0Press,\t\r \u00C2\u00A02002.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Sackley,\t\r \u00C2\u00A0C,\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0Disease:\t\r \u00C2\u00A0Current\t\r \u00C2\u00A0Epidemiological\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Pharmacological\t\r \u00C2\u00A0Management\t\r \u00C2\u00A0in\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0Primary\t\r \u00C2\u00A0Care.\"\t\r \u00C2\u00A0Neuroepidemiology,\t\r \u00C2\u00A02011:\t\r \u00C2\u00A0216-\u00C2\u00AD\u00E2\u0080\u0090221.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Sandra\t\r \u00C2\u00A0Close\t\r \u00C2\u00A0Kirkwood,\t\r \u00C2\u00A0Jessica\t\r \u00C2\u00A0L.\t\r \u00C2\u00A0Su,\t\r \u00C2\u00A0P.\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0Conneally,\t\r \u00C2\u00A0Tatiana\t\r \u00C2\u00A0Foroud.\t\r \u00C2\u00A0\"Progression\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Symptoms\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Early\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Middle\t\r \u00C2\u00A0Stages\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Neurol\t\r \u00C2\u00A058\t\r \u00C2\u00A0(2001):\t\r \u00C2\u00A0273-\u00C2\u00AD\u00E2\u0080\u0090278.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Scrimgeour\t\r \u00C2\u00A0EM,\t\r \u00C2\u00A0Pfumojena\t\r \u00C2\u00A0JW.\t\r \u00C2\u00A0\"Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0black\t\r \u00C2\u00A0Zimbabwean\t\r \u00C2\u00A0families\t\r \u00C2\u00A0living\t\r \u00C2\u00A0near\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Mozambique\t\r \u00C2\u00A0border.\"\t\r \u00C2\u00A0Am\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Med\t\r \u00C2\u00A0Genet\t\r \u00C2\u00A06\t\r \u00C2\u00A0(1992):\t\r \u00C2\u00A0762-\u00C2\u00AD\u00E2\u0080\u0090766.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Shiwach\t\r \u00C2\u00A0RS,\t\r \u00C2\u00A0Lindenbaum\t\r \u00C2\u00A0RH.\t\r \u00C2\u00A0\"Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0among\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0immigrants\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Indian\t\r \u00C2\u00A0subcontinent.\"\t\r \u00C2\u00A0Br\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A01990:\t\r \u00C2\u00A0598-\u00C2\u00AD\u00E2\u0080\u0090599.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 89\t\r \u00C2\u00A0 Shiwach,\t\r \u00C2\u00A0RS.\t\r \u00C2\u00A0\"Prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0region.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0 of\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A01994:\t\r \u00C2\u00A0414-\u00C2\u00AD\u00E2\u0080\u0090415.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Shokeir,\t\r \u00C2\u00A0MHK.\t\r \u00C2\u00A0\"Investigations\t\r \u00C2\u00A0on\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Canadian\t\r \u00C2\u00A0Prairies\t\r \u00C2\u00A01.\t\r \u00C2\u00A0Prevalence\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01975:\t\r \u00C2\u00A0345-\u00C2\u00AD\u00E2\u0080\u0090348.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Simpson,\t\r \u00C2\u00A0John.\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0English\t\r \u00C2\u00A0Dictionary.\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0University\t\r \u00C2\u00A0Press,\t\r \u00C2\u00A01989.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Simpson,\t\r \u00C2\u00A0SA,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0AW\t\r \u00C2\u00A0Johnston.\t\r \u00C2\u00A0\"The\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0patterns\t\r \u00C2\u00A0of\t\r \u00C2\u00A0care\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Grampian.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Psychiatry,\t\r \u00C2\u00A01989:\t\r \u00C2\u00A0799-\u00C2\u00AD\u00E2\u0080\u0090804.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 StatisticsCanada.\t\r \u00C2\u00A0Death\t\r \u00C2\u00A0by\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0.\t\r \u00C2\u00A02008.\t\r \u00C2\u00A0http://www.statcan.gc.ca/pub/84-\u00C2\u00AD\u00E2\u0080\u0090208-\u00C2\u00AD\u00E2\u0080\u0090x/2011001/tbl-\u00C2\u00AD\u00E2\u0080\u0090eng.htm\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A0Feb\t\r \u00C2\u00A029,\t\r \u00C2\u00A02012).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Stats,\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0Population\t\r \u00C2\u00A0Projections\t\r \u00C2\u00A02009\t\r \u00C2\u00A0to\t\r \u00C2\u00A02036.\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0Stats,\t\r \u00C2\u00A02009.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Tabrizi\t\r \u00C2\u00A0SJ,\t\r \u00C2\u00A0Douglas\t\r \u00C2\u00A0R\t\r \u00C2\u00A0Langbehn,\t\r \u00C2\u00A0Blair\t\r \u00C2\u00A0R\t\r \u00C2\u00A0Leavitt,\t\r \u00C2\u00A0Raymund\t\r \u00C2\u00A0AC\t\r \u00C2\u00A0Roos,\t\r \u00C2\u00A0Alexandra\t\r \u00C2\u00A0Durr,\t\r \u00C2\u00A0David\t\r \u00C2\u00A0Craufurd,\t\r \u00C2\u00A0Christopher\t\r \u00C2\u00A0Kennard,\t\r \u00C2\u00A0Stephen\t\r \u00C2\u00A0L\t\r \u00C2\u00A0Hicks,\t\r \u00C2\u00A0Nick\t\r \u00C2\u00A0C\t\r \u00C2\u00A0Fox,\t\r \u00C2\u00A0Rachael\t\r \u00C2\u00A0I\t\r \u00C2\u00A0Scahill,\t\r \u00C2\u00A0Beth\t\r \u00C2\u00A0Borowsky,\t\r \u00C2\u00A0Allan\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Tobin,\t\r \u00C2\u00A0H\t\r \u00C2\u00A0Diana\t\r \u00C2\u00A0Rosas,\t\r \u00C2\u00A0Hans\t\r \u00C2\u00A0Johnson,\t\r \u00C2\u00A0Ralf\t\r \u00C2\u00A0Reilmann,\t\r \u00C2\u00A0Bernhard\t\r \u00C2\u00A0Landwehrmeyer,\t\r \u00C2\u00A0Julie\t\r \u00C2\u00A0C\t\r \u00C2\u00A0Stout,\t\r \u00C2\u00A0the\t\r \u00C2\u00A0TRACK-\u00C2\u00AD\u00E2\u0080\u0090HD\t\r \u00C2\u00A0investigators.\t\r \u00C2\u00A0\u00E2\u0080\u009CBiological\t\r \u00C2\u00A0and\t\r \u00C2\u00A0clinicalmanifestations\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0longitudinal\t\r \u00C2\u00A0TRACK-\u00C2\u00AD\u00E2\u0080\u0090HD\t\r \u00C2\u00A0study:\t\r \u00C2\u00A0cross-\u00C2\u00AD\u00E2\u0080\u0090sectional\t\r \u00C2\u00A0analysis\t\r \u00C2\u00A0of\t\r \u00C2\u00A0baseline\t\r \u00C2\u00A0data\u00E2\u0080\u009D.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Lancet\t\r \u00C2\u00A0Neurology.\t\r \u00C2\u00A02009.\t\r \u00C2\u00A08(9):\t\r \u00C2\u00A0791-\u00C2\u00AD\u00E2\u0080\u0090801\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Tassicker\t\r \u00C2\u00A0RJ,\t\r \u00C2\u00A0Betty\t\r \u00C2\u00A0Teltscher,\t\r \u00C2\u00A0M\t\r \u00C2\u00A0Kaye\t\r \u00C2\u00A0Trembath,\t\r \u00C2\u00A0Veronica\t\r \u00C2\u00A0Collins,\t\r \u00C2\u00A0Leslie\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Sheffield,\t\r \u00C2\u00A0Edmond\t\r \u00C2\u00A0Chiu,\t\r \u00C2\u00A0Lyle\t\r \u00C2\u00A0Gurrin6\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Martin\t\r \u00C2\u00A0B\t\r \u00C2\u00A0Delatycki.\t\r \u00C2\u00A0\"Problems\t\r \u00C2\u00A0assessing\t\r \u00C2\u00A0uptake\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0proposed\t\r \u00C2\u00A0solution.\"\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A02009:\t\r \u00C2\u00A066\u00E2\u0080\u009370.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Taylor,\t\r \u00C2\u00A0SD.\t\r \u00C2\u00A0\"Demand\t\r \u00C2\u00A0for\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0testing\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Australia.\"\t\r \u00C2\u00A0 Medical\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Australia,\t\r \u00C2\u00A01994:\t\r \u00C2\u00A0354\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0355.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 TheHuntington\u00E2\u0080\u0099sDiseaseCollaborativeResearchGroup.\t\r \u00C2\u00A0\"A\t\r \u00C2\u00A0novel\t\r \u00C2\u00A0gene\t\r \u00C2\u00A0containing\t\r \u00C2\u00A0a\t\r \u00C2\u00A0trinucleotide\t\r \u00C2\u00A0repeat\t\r \u00C2\u00A0that\t\r \u00C2\u00A0is\t\r \u00C2\u00A0expanded\t\r \u00C2\u00A0and\t\r \u00C2\u00A0unstable\t\r \u00C2\u00A0on\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0chromo-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0somes.\"\t\r \u00C2\u00A0Cell\t\r \u00C2\u00A072\t\r \u00C2\u00A0(1993):\t\r \u00C2\u00A0971\u00E2\u0080\u0093983.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 TheProvinceofBritishColumbia.\t\r \u00C2\u00A0Vital\t\r \u00C2\u00A0Statistics\t\r \u00C2\u00A0Agency\t\r \u00C2\u00A0BC.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.vs.gov.bc.ca/.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Therapeutics,\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Molecular\t\r \u00C2\u00A0Medicine\t\r \u00C2\u00A0and.\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease.\t\r \u00C2\u00A02011.\t\r \u00C2\u00A0http://www.cmmt.ubc.ca/outreach/hd-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0(accessed\t\r \u00C2\u00A002\t\r \u00C2\u00A002,\t\r \u00C2\u00A02011).\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Walker,\t\r \u00C2\u00A0DA,\t\r \u00C2\u00A0PS\t\r \u00C2\u00A0Harper,\t\r \u00C2\u00A0CE\t\r \u00C2\u00A0Wells,\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Tyler,\t\r \u00C2\u00A0K\t\r \u00C2\u00A0Davies,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0RG\t\r \u00C2\u00A0Newcombe.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0Chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales.\t\r \u00C2\u00A0A\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0and\t\r \u00C2\u00A0epidemiological\t\r \u00C2\u00A0study.\"\t\r \u00C2\u00A0Clinical\t\r \u00C2\u00A0Genetics,\t\r \u00C2\u00A01981:\t\r \u00C2\u00A0213-\u00C2\u00AD\u00E2\u0080\u0090221.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Walker,\t\r \u00C2\u00A0Francis\t\r \u00C2\u00A0O.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0Lancet\t\r \u00C2\u00A0369\t\r \u00C2\u00A0(2007):\t\r \u00C2\u00A0218-\u00C2\u00AD\u00E2\u0080\u0090228.\t\r \u00C2\u00A0Wallace,\t\r \u00C2\u00A0D.\t\r \u00C2\u00A0C.\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Parker,\t\r \u00C2\u00A0N.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Queensland:The\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0story\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Advances\t\r \u00C2\u00A0in\t\r \u00C2\u00A0 Neurology\t\r \u00C2\u00A01\t\r \u00C2\u00A0(1973):\t\r \u00C2\u00A0223-\u00C2\u00AD\u00E2\u0080\u0090236.\t\r \u00C2\u00A0\t\r \u00C2\u00A0Warby\t\r \u00C2\u00A0SC,\t\r \u00C2\u00A0Henk\t\r \u00C2\u00A0Visscher,\t\r \u00C2\u00A0Jennifer\t\r \u00C2\u00A0A\t\r \u00C2\u00A0Collins,\t\r \u00C2\u00A0Crystal\t\r \u00C2\u00A0N\t\r \u00C2\u00A0Doty,\t\r \u00C2\u00A0Catherine\t\r \u00C2\u00A0Carter,\t\r \u00C2\u00A0Stefanie\t\r \u00C2\u00A0L\t\r \u00C2\u00A0Butland,\t\r \u00C2\u00A0Anna\t\r \u00C2\u00A0R\t\r \u00C2\u00A0Hayden,\t\r \u00C2\u00A0Ichiro\t\r \u00C2\u00A0Kanazawa,\t\r \u00C2\u00A0Colin\t\r \u00C2\u00A0J\t\r \u00C2\u00A0Ross\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0R\t\r \u00C2\u00A0Hayden.\t\r \u00C2\u00A0\"HTT\t\r \u00C2\u00A0haplotypes\t\r \u00C2\u00A0contribute\t\r \u00C2\u00A0to\t\r \u00C2\u00A0differences\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0between\t\r \u00C2\u00A0Europe\t\r \u00C2\u00A0and\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Asia\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0 European\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0,\t\r \u00C2\u00A02011:\t\r \u00C2\u00A0561\u00E2\u0080\u0093566.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Warby\t\r \u00C2\u00A0SC,\t\r \u00C2\u00A0Alexandre\t\r \u00C2\u00A0Montpetit,\t\r \u00C2\u00A0Anna\t\r \u00C2\u00A0R.\t\r \u00C2\u00A0Hayden,\t\r \u00C2\u00A0Jeffrey\t\r \u00C2\u00A0B.\t\r \u00C2\u00A0Carroll,\t\r \u00C2\u00A0Stefanie\t\r \u00C2\u00A0L.\t\r \u00C2\u00A0Butland,\t\r \u00C2\u00A0Henk\t\r \u00C2\u00A0Visscher,\t\r \u00C2\u00A0Jennifer\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0Collins,\t\r \u00C2\u00A0Alicia\t\r \u00C2\u00A0Semaka,\t\r \u00C2\u00A0Thomas\t\r \u00C2\u00A0J.\t\r \u00C2\u00A0Hudson,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Michael\t\r \u00C2\u00A0R.\t\r \u00C2\u00A0Hayden.\t\r \u00C2\u00A0\"CAG\t\r \u00C2\u00A0Expansion\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Gene\t\r \u00C2\u00A0Is\t\r \u00C2\u00A0Associated\t\r \u00C2\u00A0with\t\r \u00C2\u00A0a\t\r \u00C2\u00A0Specific\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Targetable\t\r \u00C2\u00A0Predisposing\t\r \u00C2\u00A0Haplogroup\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0The\t\r \u00C2\u00A0American\t\r \u00C2\u00A0Journal\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Human\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A084\t\r \u00C2\u00A0(2009):\t\r \u00C2\u00A0351\u00E2\u0080\u0093366.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 90\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 WorldBank.\t\r \u00C2\u00A0Population\t\r \u00C2\u00A0ages\t\r \u00C2\u00A065\t\r \u00C2\u00A0and\t\r \u00C2\u00A0above\t\r \u00C2\u00A0(%\t\r \u00C2\u00A0of\t\r \u00C2\u00A0total).\t\r \u00C2\u00A003\t\r \u00C2\u00A027,\t\r \u00C2\u00A02012.\t\r \u00C2\u00A0http://data.worldbank.org/indicator/SP.POP.65UP.TO.ZS.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Wright,\t\r \u00C2\u00A0HH,\t\r \u00C2\u00A0CN\t\r \u00C2\u00A0Still,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0RK\t\r \u00C2\u00A0Abramson.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0black\t\r \u00C2\u00A0kindreds\t\r \u00C2\u00A0in\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Carolina.\t\r \u00C2\u00A0.\"\t\r \u00C2\u00A0Arch\t\r \u00C2\u00A0Neurol,\t\r \u00C2\u00A01981:\t\r \u00C2\u00A0412-\u00C2\u00AD\u00E2\u0080\u0090114.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \u00C3\u0098deg\u00C3\u00A5rd,\t\r \u00C2\u00A0Letten\t\r \u00C2\u00A0Saugstad\t\r \u00C2\u00A0and\t\r \u00C2\u00A0\u00C3\u0098rnulv.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0chorea\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Norway.\"\t\r \u00C2\u00A0Psychological\t\r \u00C2\u00A0 Medicine\t\r \u00C2\u00A016\t\r \u00C2\u00A0(1986):\t\r \u00C2\u00A039-\u00C2\u00AD\u00E2\u0080\u009048.\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Young\t\r \u00C2\u00A0A,\t\r \u00C2\u00A0Ira\t\r \u00C2\u00A0Shoulson,\t\r \u00C2\u00A0John\t\r \u00C2\u00A0B.\t\r \u00C2\u00A0Penney,\t\r \u00C2\u00A0Simon\t\r \u00C2\u00A0Starosta-\u00C2\u00AD\u00E2\u0080\u0090Rubinstein,\t\r \u00C2\u00A0Fidela\t\r \u00C2\u00A0Gomez,\t\r \u00C2\u00A0RN,\t\r \u00C2\u00A0Helen\t\r \u00C2\u00A0Travers,,\t\r \u00C2\u00A0Maria\t\r \u00C2\u00A0A.\t\r \u00C2\u00A0Ramos-\u00C2\u00AD\u00E2\u0080\u0090Arroyo,,\t\r \u00C2\u00A0S.\t\r \u00C2\u00A0Robert\t\r \u00C2\u00A0Snodgrass,\t\r \u00C2\u00A0Ernesto\t\r \u00C2\u00A0Bonilla,\t\r \u00C2\u00A0Humberto\t\r \u00C2\u00A0Moreno,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Nancy\t\r \u00C2\u00A0S.\t\r \u00C2\u00A0Wexler.\t\r \u00C2\u00A0\"Huntington's\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0Neurologic\t\r \u00C2\u00A0features\t\r \u00C2\u00A0and\t\r \u00C2\u00A0functional\t\r \u00C2\u00A0decline.\"\t\r \u00C2\u00A0 Neurology\t\r \u00C2\u00A036,\t\r \u00C2\u00A0no.\t\r \u00C2\u00A02\t\r \u00C2\u00A0(1986):\t\r \u00C2\u00A0224.\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 91\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A01a\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Worldwide\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0on\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0from\t\r \u00C2\u00A01940-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0\t\r \u00C2\u00A0 POPULATION\t\r \u00C2\u00A0 DATE\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0SURVEY\t\r \u00C2\u00A0 PREVALENCE\t\r \u00C2\u00A0 (#/100,000)\t\r \u00C2\u00A0 REFERENCE\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(New\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales)\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0(McCusker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02000)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Queensland)\t\r \u00C2\u00A0 1976\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A0 (Wallace\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Parker\t\r \u00C2\u00A01979)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Queensland)\t\r \u00C2\u00A0 1969\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 (Wallace\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Parker\t\r \u00C2\u00A01973)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Tasmania)\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 12.1\t\r \u00C2\u00A0 (Pridmore\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Victoria)\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0 (Brothers\t\r \u00C2\u00A01964)\t\r \u00C2\u00A0Austria\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 (Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Belgium\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1970\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0 (Husquinet\t\r \u00C2\u00A01973)\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0(Quebec)\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0 (Barbeau\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01964)\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0(Manitoba\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Saskatchewan)\t\r \u00C2\u00A0 1975\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 (Shokeir\t\r \u00C2\u00A01975)\t\r \u00C2\u00A0China\t\r \u00C2\u00A0(Hong\t\r \u00C2\u00A0Kong)\t\r \u00C2\u00A0 1984-\u00C2\u00AD\u00E2\u0080\u00901991\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 (Leung\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01992)\t\r \u00C2\u00A0China\t\r \u00C2\u00A0(Hong\t\r \u00C2\u00A0Kong)\t\r \u00C2\u00A0 1984-\u00C2\u00AD\u00E2\u0080\u00901991\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 (Chang\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0Croatia\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 (Hecimovic\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02002)\t\r \u00C2\u00A0Egypt\t\r \u00C2\u00A0(Assiut)\t\r \u00C2\u00A0 1988-\u00C2\u00AD\u00E2\u0080\u00901990\t\r \u00C2\u00A0 21\t\r \u00C2\u00A0 (Kandil\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0Finland\t\r \u00C2\u00A0 1986\t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 (Palo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01987)\t\r \u00C2\u00A0France\t\r \u00C2\u00A0(North-\u00C2\u00AD\u00E2\u0080\u0090West)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 (Petit\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Salomez\t\r \u00C2\u00A01985)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0(Kassel)\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 2.6\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0(Rhineland)\t\r \u00C2\u00A0 1933\t\r \u00C2\u00A0 3.2\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0(West,\t\r \u00C2\u00A0without\t\r \u00C2\u00A0Berlin\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Saarland)\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 2.2\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0(West,\t\r \u00C2\u00A0without\t\r \u00C2\u00A0Berlin\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Saarland)\t\r \u00C2\u00A0 1939\t\r \u00C2\u00A0 2.2\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 (Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Greece\t\r \u00C2\u00A0 2004\t\r \u00C2\u00A0 3.95\t\r \u00C2\u00A0 (Panas\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0Guam\t\r \u00C2\u00A0(Chamorros)\t\r \u00C2\u00A0 1967\t\r \u00C2\u00A0 0\t\r \u00C2\u00A0 (Chen\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01968)\t\r \u00C2\u00A0Iceland\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 2.7\t\r \u00C2\u00A0 (Gudmundsson\t\r \u00C2\u00A01969)\t\r \u00C2\u00A0India\t\r \u00C2\u00A0(Pakistan,\t\r \u00C2\u00A0Punjab\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Gujerat)\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 (Shiwach\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Lindenbaum\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Aosta)\t\r \u00C2\u00A0 1982-\u00C2\u00AD\u00E2\u0080\u00901991\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Emilia\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Parma)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 4.8\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Ferrara\t\r \u00C2\u00A0province)\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 1.9\t\r \u00C2\u00A0 (Govoni\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01988)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Florence)\t\r \u00C2\u00A0 1970-\u00C2\u00AD\u00E2\u0080\u00901979\t\r \u00C2\u00A0 4.1\t\r \u00C2\u00A0 (Groppi\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01986)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Frogiuone)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 2.6\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Genoa\t\r \u00C2\u00A0Region)\t\r \u00C2\u00A0 1930-\u00C2\u00AD\u00E2\u0080\u00901977\t\r \u00C2\u00A0 28\t\r \u00C2\u00A0 (Roccatagliata\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01979)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Genoa-\u00C2\u00AD\u00E2\u0080\u0090Savona)\t\r \u00C2\u00A0 1973\t\r \u00C2\u00A0 4.5\t\r \u00C2\u00A0 (Roccatagliata\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Albano\t\r \u00C2\u00A01976)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Latima)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Lazio)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 2.6\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Puglia)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 2.9\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Rieti)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Rome)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 2.5\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Tuscany)\t\r \u00C2\u00A0 1978\t\r \u00C2\u00A0 2.3\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Viterbo)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 1.5\t\r \u00C2\u00A0 (Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0(Aichi)\t\r \u00C2\u00A0 1959\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 (Kishimoto\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01957)\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0(Ibaraki)\t\r \u00C2\u00A0 1982\t\r \u00C2\u00A0 0.1\t\r \u00C2\u00A0 (Kanazawa\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0(San-\u00C2\u00AD\u00E2\u0080\u0090in)\t\r \u00C2\u00A0 1993\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A0 (Nakashima\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01996)\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0(San-\u00C2\u00AD\u00E2\u0080\u0090in)\t\r \u00C2\u00A0 1997\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A0 (Adachi\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Nakashima\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Malta\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 11.8\t\r \u00C2\u00A0 (Gassivaro\t\r \u00C2\u00A0Gallo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Malta\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 7.8\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Mauritius\t\r \u00C2\u00A0(European\t\r \u00C2\u00A0descent)\t\r \u00C2\u00A0 1977\t\r \u00C2\u00A0 46.2\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01981)\t\r \u00C2\u00A0Mexico\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 (Alonso\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02009)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 92\t\r \u00C2\u00A0 New\t\r \u00C2\u00A0Zealand\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 (Harper\t\r \u00C2\u00A01996)\t\r \u00C2\u00A0Nigeria\t\r \u00C2\u00A0(Ibadan)\t\r \u00C2\u00A0 1984\t\r \u00C2\u00A0 0.2\t\r \u00C2\u00A0 (Aiyesimoju\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01984)\t\r \u00C2\u00A0Norway\t\r \u00C2\u00A0 1930\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 (Saugstad\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Odeg\u00C3\u00A5rd\t\r \u00C2\u00A01986)\t\r \u00C2\u00A0Norway\t\r \u00C2\u00A0 1940\t\r \u00C2\u00A0 6.7\t\r \u00C2\u00A0 (Saugstad\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Odeg\u00C3\u00A5rd\t\r \u00C2\u00A01986)\t\r \u00C2\u00A0Norway\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A0 (Saugstad\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Odeg\u00C3\u00A5rd\t\r \u00C2\u00A01986)\t\r \u00C2\u00A0Poland\t\r \u00C2\u00A0(Pruszkow)\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 4.8\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Russia\t\r \u00C2\u00A0(6\t\r \u00C2\u00A0populations\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Central\t\r \u00C2\u00A0Asia)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1.3\t\r \u00C2\u00A0 (Kirilenko\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02004)\t\r \u00C2\u00A0Russia\t\r \u00C2\u00A0(Volgograd\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Volzhsky)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 0.6\t\r \u00C2\u00A0 (Kirilenko\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02004)\t\r \u00C2\u00A0Russia\t\r \u00C2\u00A0(Vladimir\t\r \u00C2\u00A0Oblast)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1.9\t\r \u00C2\u00A0 (Kirilenko\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02004)\t\r \u00C2\u00A0Slovenia\t\r \u00C2\u00A0 2006\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 (Peterlin\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02008)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 0.6\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01982)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 0.01\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01980)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0(Cape\t\r \u00C2\u00A0Coloured,\t\r \u00C2\u00A0mixed\t\r \u00C2\u00A0race)\t\r \u00C2\u00A0 1976\t\r \u00C2\u00A0 3.5\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A01977)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0(Mixed\t\r \u00C2\u00A0race)\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 0.9\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A01977)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0(White\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Coloured\t\r \u00C2\u00A0population)\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 2.2\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01980)\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0(Whites\t\r \u00C2\u00A0Afrikaans)\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A0 (Hayden\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A01977)\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0(Salamanca)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 (Ruiz\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01985)\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0(Cadiz\t\r \u00C2\u00A0province)\t\r \u00C2\u00A0 1968\t\r \u00C2\u00A0 1.3\t\r \u00C2\u00A0 (Calcedo\t\r \u00C2\u00A0Ord\u00C3\u00B3ez\t\r \u00C2\u00A01970)\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0(Valencia)\t\r \u00C2\u00A0 1987-\u00C2\u00AD\u00E2\u0080\u00901992\t\r \u00C2\u00A0 5.4\t\r \u00C2\u00A0 (Burguera\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01997)\t\r \u00C2\u00A0Sweden\t\r \u00C2\u00A0 1965\t\r \u00C2\u00A0 4.7\t\r \u00C2\u00A0 (Mattsson\t\r \u00C2\u00A01974)\t\r \u00C2\u00A0Sweden\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0Switzerland\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 (Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01999)\t\r \u00C2\u00A0Taiwan\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 0.42\t\r \u00C2\u00A0 (Chen\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010)\t\r \u00C2\u00A0Tanzania\t\r \u00C2\u00A0(Mount\t\r \u00C2\u00A0Kilimanjaro,\t\r \u00C2\u00A0Bantu)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 (Scrimgeour\t\r \u00C2\u00A01981)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Carlisle)\t\r \u00C2\u00A0 1961\t\r \u00C2\u00A0 2.8\t\r \u00C2\u00A0 (Brewis\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01966)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Cornwall)\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A0 (Bickford\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Ellison\t\r \u00C2\u00A01953)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Cornwall)\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 4.9\t\r \u00C2\u00A0 (Harper\t\r \u00C2\u00A01996)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Devon)\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0 (Harper\t\r \u00C2\u00A01996)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Anglia)\t\r \u00C2\u00A0 1971\t\r \u00C2\u00A0 9.19\t\r \u00C2\u00A0 (Caro\t\r \u00C2\u00A01977)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Essex)\t\r \u00C2\u00A0 1965\t\r \u00C2\u00A0 2.5\t\r \u00C2\u00A0 (Heathfield\t\r \u00C2\u00A01967)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Leeds)\t\r \u00C2\u00A0 1966\t\r \u00C2\u00A0 4.2\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Northamptonshire)\t\r \u00C2\u00A0 1967-\u00C2\u00AD\u00E2\u0080\u00901968\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 (Oliver\t\r \u00C2\u00A01970)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Northamptonshire)\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0 (Reid\t\r \u00C2\u00A01960)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Northamptonshire)\t\r \u00C2\u00A0 1954-\u00C2\u00AD\u00E2\u0080\u00901955\t\r \u00C2\u00A0 6.5\t\r \u00C2\u00A0 (Pleydell\t\r \u00C2\u00A01955)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0region)\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 (Shiwach\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Somerset)\t\r \u00C2\u00A0 1965\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(England,\t\r \u00C2\u00A0Wessex)\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 3.7\t\r \u00C2\u00A0 (Harper\t\r \u00C2\u00A01996)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Indian\t\r \u00C2\u00A0subcontinent\t\r \u00C2\u00A0immigrants)\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 (Shiwach\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Lindenbaum\t\r \u00C2\u00A01990)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Ireland,\t\r \u00C2\u00A0Northern)\t\r \u00C2\u00A0 1991\t\r \u00C2\u00A0 6.4\t\r \u00C2\u00A0 (Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01995)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Ireland,\t\r \u00C2\u00A0Northern,\t\r \u00C2\u00A0County\t\r \u00C2\u00A0Donegal)\t\r \u00C2\u00A0 1991\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0 (Morrison\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Nevin\t\r \u00C2\u00A01993)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Scotland,\t\r \u00C2\u00A0Grampian,\t\r \u00C2\u00A0north-\u00C2\u00AD\u00E2\u0080\u0090east\t\r \u00C2\u00A0)\t\r \u00C2\u00A0 1984\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 (Simpson\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Johnston\t\r \u00C2\u00A01989)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Scotland,\t\r \u00C2\u00A0South-\u00C2\u00AD\u00E2\u0080\u0090East)\t\r \u00C2\u00A0 1967\t\r \u00C2\u00A0 7.2\t\r \u00C2\u00A0 (Cameron\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Venters\t\r \u00C2\u00A01967)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Scotland,\t\r \u00C2\u00A0West)\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 (Bolt\t\r \u00C2\u00A01970)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales,\t\r \u00C2\u00A0North)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0 (Quarrell\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01988)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales,\t\r \u00C2\u00A0South\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Glamorgan)\t\r \u00C2\u00A0 1988\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 (MacMillan\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A01991)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales)\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 6.2\t\r \u00C2\u00A0 (James\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales,\t\r \u00C2\u00A0South)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 8.9\t\r \u00C2\u00A0 (Quarrell\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01988)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales,\t\r \u00C2\u00A0South)\t\r \u00C2\u00A0 1971\t\r \u00C2\u00A0 7.6\t\r \u00C2\u00A0 (Walker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01981)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0(Wales,\t\r \u00C2\u00A0South)\t\r \u00C2\u00A0 1971\t\r \u00C2\u00A0 7.6\t\r \u00C2\u00A0 (Harper\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01979)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 6.25\t\r \u00C2\u00A0 (Sackley\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02011)\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 12.4\t\r \u00C2\u00A0 (Rawlins,\t\r \u00C2\u00A02010)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 93\t\r \u00C2\u00A0 USA\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0(Whites)\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(African\t\r \u00C2\u00A0Americans)\t\r \u00C2\u00A0 1940\t\r \u00C2\u00A0 1.5\t\r \u00C2\u00A0 (Reed\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Chandler\t\r \u00C2\u00A01958)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Maryland)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 (Folstein\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01987)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Maryland,\t\r \u00C2\u00A0African\t\r \u00C2\u00A0Americans)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 6.4\t\r \u00C2\u00A0 (Folstein\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01987)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Michigan)\t\r \u00C2\u00A0 1940\t\r \u00C2\u00A0 4.1\t\r \u00C2\u00A0 (Reed\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Chandler\t\r \u00C2\u00A01958)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Minnesota)\t\r \u00C2\u00A0 1955\t\r \u00C2\u00A0 5.4\t\r \u00C2\u00A0 (Pearson\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01955)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Minnesota,\t\r \u00C2\u00A0Olmsted\t\r \u00C2\u00A0county)\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 (Kokmen\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Minnesota,\t\r \u00C2\u00A0Olmsted\t\r \u00C2\u00A0county)\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 (Kokmen\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01994)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(Minnesota,\t\r \u00C2\u00A0Rochester)\t\r \u00C2\u00A0 1955\t\r \u00C2\u00A0 6.7\t\r \u00C2\u00A0 (Kurland\t\r \u00C2\u00A01958)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(New\t\r \u00C2\u00A0York)\t\r \u00C2\u00A0 1973\t\r \u00C2\u00A0 3.5\t\r \u00C2\u00A0 (Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02001)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(South\t\r \u00C2\u00A0Carolina)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 (Wright\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01981)\t\r \u00C2\u00A0USA\t\r \u00C2\u00A0(South\t\r \u00C2\u00A0Carolina,\t\r \u00C2\u00A0White)\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 4.8\t\r \u00C2\u00A0 (Wright\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01981)\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 (Paradisi\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A02008)\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0(Lake\t\r \u00C2\u00A0Maracaibo)\t\r \u00C2\u00A0 1973\t\r \u00C2\u00A0 699.2\t\r \u00C2\u00A0 (Avila-\u00C2\u00AD\u00E2\u0080\u0090Giron\t\r \u00C2\u00A01973)\t\r \u00C2\u00A0Yugoslavia\t\r \u00C2\u00A0(Rijeka\t\r \u00C2\u00A0district)\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 4.5\t\r \u00C2\u00A0 (Sepci\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al\t\r \u00C2\u00A01989)\t\r \u00C2\u00A0Zimbabwe\t\r \u00C2\u00A0(Manicaland\t\r \u00C2\u00A0region,\t\r \u00C2\u00A0Shona)\t\r \u00C2\u00A0 1988-\u00C2\u00AD\u00E2\u0080\u00901989\t\r \u00C2\u00A0 0.8\t\r \u00C2\u00A0 (Scrimgeour\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Pfumojena\t\r \u00C2\u00A01992)\t\r \u00C2\u00A0 Global\t\r \u00C2\u00A0Average\t\r \u00C2\u00A0(including\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Maracaibo)\t\r \u00C2\u00A0 1930-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0 11.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Global\t\r \u00C2\u00A0Average\t\r \u00C2\u00A0(excluding\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Maracaibo)\t\r \u00C2\u00A0 1930-\u00C2\u00AD\u00E2\u0080\u00902010\t\r \u00C2\u00A0 5.1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 94\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A01b\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Worldwide\t\r \u00C2\u00A0reports\t\r \u00C2\u00A0on\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0prevalence,\t\r \u00C2\u00A0incidence\t\r \u00C2\u00A0and\t\r \u00C2\u00A0population\t\r \u00C2\u00A0at\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0by\t\r \u00C2\u00A0region\t\r \u00C2\u00A0 REGION\t\r \u00C2\u00A0 YEAR\t\r \u00C2\u00A0OF\t\r \u00C2\u00A0REPORT\t\r \u00C2\u00A0 PREVALENCE\t\r \u00C2\u00A0(#/100,000)\t\r \u00C2\u00A0 INCIDENCE\t\r \u00C2\u00A0(#/million/year)\t\r \u00C2\u00A0 POP\t\r \u00C2\u00A0AT-\u00C2\u00AD\u00E2\u0080\u0090RISK\t\r \u00C2\u00A0(#/100,000)\t\r \u00C2\u00A0 REFERENCE\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0Americas\t\r \u00C2\u00A0Canada\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Quebec\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Barbeau\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01964\t\r \u00C2\u00A0Prairies\t\r \u00C2\u00A0(Saskatchewan\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Manitoba \t\r \u00C2\u00A01975\t\r \u00C2\u00A0 \t\r \u00C2\u00A08.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Shokeir\t\r \u00C2\u00A01975\t\r \u00C2\u00A0British\t\r \u00C2\u00A0Columbia\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00902000\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 42.0\t\r \u00C2\u00A0 Almqvist\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001,\t\r \u00C2\u00A0Creighton\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02003\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5.9\t\r \u00C2\u00A0 6.9\t\r \u00C2\u00A0 42.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0United\t\r \u00C2\u00A0States\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Michigan\t\r \u00C2\u00A0 1940\t\r \u00C2\u00A0 1.5\t\r \u00C2\u00A04.1\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Reed\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Chandler,\t\r \u00C2\u00A01958\t\r \u00C2\u00A0Maryland\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 6.4\t\r \u00C2\u00A05.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Folstein\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01987\t\r \u00C2\u00A0Minnesota\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1950-\u00C2\u00AD\u00E2\u0080\u00901989\t\r \u00C2\u00A0 2.0\t\r \u00C2\u00A0 2.0-\u00C2\u00AD\u00E2\u0080\u00904.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Folstein\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01987\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Carolina\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A04.8\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Wright\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01980\t\r \u00C2\u00A0New\t\r \u00C2\u00A0York\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1973\t\r \u00C2\u00A0 3.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3.6\t\r \u00C2\u00A0 3.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mexico\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mexico\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 4.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Alonso\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02009\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0\t\r \u00C2\u00A0Venezuela\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Paradisi\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02008\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Maracaibo\t\r \u00C2\u00A0 1973\t\r \u00C2\u00A0 699\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Avila-\u00C2\u00AD\u00E2\u0080\u0090Giron,\t\r \u00C2\u00A01973\t\r \u00C2\u00A0 Europe\t\r \u00C2\u00A0The\t\r \u00C2\u00A0United\t\r \u00C2\u00A0Kingdom\t\r \u00C2\u00A0Carlisle\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1961\t\r \u00C2\u00A0 2.8\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Brewis\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01966\t\r \u00C2\u00A0Cornwall\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A01987\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A04.9\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Bickford\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Ellison,\t\r \u00C2\u00A01953,\t\r \u00C2\u00A0Harper\t\r \u00C2\u00A01996\t\r \u00C2\u00A0Devon\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Harper\t\r \u00C2\u00A01996\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Anglia\t\r \u00C2\u00A0 1971\t\r \u00C2\u00A0 9.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Caro\t\r \u00C2\u00A01977\t\r \u00C2\u00A0Essex\t\r \u00C2\u00A0 1965\t\r \u00C2\u00A0 2.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Heathfield,\t\r \u00C2\u00A01967\t\r \u00C2\u00A0Leeds\t\r \u00C2\u00A0 1966\t\r \u00C2\u00A0 4.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001\t\r \u00C2\u00A0Northamptonshire\t\r \u00C2\u00A0 1967-\u00C2\u00AD\u00E2\u0080\u00901968\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Oliver\t\r \u00C2\u00A01970\t\r \u00C2\u00A0Oxford\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Shiwach\t\r \u00C2\u00A01994\t\r \u00C2\u00A0Wessex\t\r \u00C2\u00A0 1987\t\r \u00C2\u00A0 3.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Harper\t\r \u00C2\u00A01996\t\r \u00C2\u00A0Somerset\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1965\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0 1991\t\r \u00C2\u00A0 6.4\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01995\t\r \u00C2\u00A0County\t\r \u00C2\u00A0Donegal\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 1991\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Morrison\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Nevin\t\r \u00C2\u00A01993\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 95\t\r \u00C2\u00A0 Ireland\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Ireland\t\r \u00C2\u00A0 2001\t\r \u00C2\u00A0 10.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 44.9\t\r \u00C2\u00A0 Morrison\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02010\t\r \u00C2\u00A0Grampian\t\r \u00C2\u00A0 1984\t\r \u00C2\u00A0 10.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Simpson\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Johnston,\t\r \u00C2\u00A01989\t\r \u00C2\u00A0South\t\r \u00C2\u00A0East\t\r \u00C2\u00A0Scotland\t\r \u00C2\u00A0 1967 7.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Cameron\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Venters,\t\r \u00C2\u00A01967\t\r \u00C2\u00A0West\t\r \u00C2\u00A0Scotland\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Bolt,\t\r \u00C2\u00A01970\t\r \u00C2\u00A0Northern\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 5.5\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Quarell\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01988\t\r \u00C2\u00A0South\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Glamorgan\t\r \u00C2\u00A0 1988\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A06.2\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Macmillan\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Harper,\t\r \u00C2\u00A01991,\t\r \u00C2\u00A0James\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01994\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 8.9\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Quarell\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01988\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 1971\t\r \u00C2\u00A0 7.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Walker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01981\t\r \u00C2\u00A0UK\t\r \u00C2\u00A0 2008\t\r \u00C2\u00A0 5.9-\u00C2\u00AD\u00E2\u0080\u00906.5\t\r \u00C2\u00A0 4.4-\u00C2\u00AD\u00E2\u0080\u00907.8\t\r \u00C2\u00A0 37.5\t\r \u00C2\u00A0 Sackley\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011\t\r \u00C2\u00A0England\t\r \u00C2\u00A0&\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 2010\t\r \u00C2\u00A0 12.4\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Rawlins\t\r \u00C2\u00A02010\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 6.1\t\r \u00C2\u00A0 41.2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Non-\u00C2\u00AD\u00E2\u0080\u0090UK\t\r \u00C2\u00A0countries\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Belgium\t\r \u00C2\u00A0 1970\t\r \u00C2\u00A0 1.6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Husquinet,\t\r \u00C2\u00A01973\t\r \u00C2\u00A0Croatia\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Hecimovic\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002\t\r \u00C2\u00A0France\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 5.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 25.0\t\r \u00C2\u00A0 Petit\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Salmonez,\t\r \u00C2\u00A01985,\t\r \u00C2\u00A0Goizet\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02002\t\r \u00C2\u00A0Germany\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 30.0\t\r \u00C2\u00A0 Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999\t\r \u00C2\u00A0Greece\t\r \u00C2\u00A0 1995-\u00C2\u00AD\u00E2\u0080\u00902008\t\r \u00C2\u00A0 2.5-\u00C2\u00AD\u00E2\u0080\u00905.4\t\r \u00C2\u00A0 2.2-\u00C2\u00AD\u00E2\u0080\u00904.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Panas\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02011\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0 1981\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Frontali\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01990\t\r \u00C2\u00A0Italy\t\r \u00C2\u00A0(Ferrara)\t\r \u00C2\u00A0 1871-\u00C2\u00AD\u00E2\u0080\u00901987\t\r \u00C2\u00A0 0.36-\u00C2\u00AD\u00E2\u0080\u00903.1\t\r \u00C2\u00A0 2.0 \t\r \u00C2\u00A0 Giovani\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01988\t\r \u00C2\u00A0Malta 1994\t\r \u00C2\u00A0 11.8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Gasivaro-\u00C2\u00AD\u00E2\u0080\u0090Gallo\t\r \u00C2\u00A01990\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0 2002\t\r \u00C2\u00A0 6.5\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 32.5\t\r \u00C2\u00A0 Maat-\u00C2\u00AD\u00E2\u0080\u0090Kievit\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000\t\r \u00C2\u00A0Norway\t\r \u00C2\u00A0 1950\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Saugstad\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Odegard\t\r \u00C2\u00A01986\t\r \u00C2\u00A0Poland\t\r \u00C2\u00A0 1960\t\r \u00C2\u00A0 4.8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001\t\r \u00C2\u00A0Russia\t\r \u00C2\u00A0 2004\t\r \u00C2\u00A0 1.9\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Kirilenko\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02004\t\r \u00C2\u00A0Slovenia\t\r \u00C2\u00A0 2006\t\r \u00C2\u00A0 5.2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Peterlin\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02008\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 8.4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Ruiz\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01985\t\r \u00C2\u00A0Spain\t\r \u00C2\u00A0 1994-\u00C2\u00AD\u00E2\u0080\u00902002\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4.7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Ramos-\u00C2\u00AD\u00E2\u0080\u0090Arroyo\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02004\t\r \u00C2\u00A0Sweden\t\r \u00C2\u00A0 1985\t\r \u00C2\u00A0 5.6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Al-\u00C2\u00AD\u00E2\u0080\u0090Jader\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02001\t\r \u00C2\u00A0Switzerland\t\r \u00C2\u00A0 1993-\u00C2\u00AD\u00E2\u0080\u00901997\t\r \u00C2\u00A0 10.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Laccone\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01999\t\r \u00C2\u00A0Iceland\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 2.7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Gudmundsson\t\r \u00C2\u00A01969\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5.4\t\r \u00C2\u00A0 3.4\t\r \u00C2\u00A0 29.2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Africa\t\r \u00C2\u00A0Egypt\t\r \u00C2\u00A0 1988-\u00C2\u00AD\u00E2\u0080\u00901990\t\r \u00C2\u00A0 21.0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Kandil\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01994\t\r \u00C2\u00A0Zimbabwe\t\r \u00C2\u00A0 1988-\u00C2\u00AD\u00E2\u0080\u00901989\t\r \u00C2\u00A0 0.8 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Scrimgeour\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Pfumojena\t\r \u00C2\u00A01992\t\r \u00C2\u00A0Tanzania\t\r \u00C2\u00A0 1980\t\r \u00C2\u00A0 7.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Scrimgeour\t\r \u00C2\u00A01981\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 96\t\r \u00C2\u00A0 Rows\t\r \u00C2\u00A0highlighted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0pink\t\r \u00C2\u00A0comprise\t\r \u00C2\u00A0studies\t\r \u00C2\u00A0performed\t\r \u00C2\u00A0after\t\r \u00C2\u00A0the\t\r \u00C2\u00A0advent\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0test.\t\r \u00C2\u00A0Numbers\t\r \u00C2\u00A0in\t\r \u00C2\u00A0light\t\r \u00C2\u00A0grey\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0due\t\r \u00C2\u00A0to\t\r \u00C2\u00A0methodological\t\r \u00C2\u00A0inaccuracies.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0 1979\t\r \u00C2\u00A0 2.2\t\r \u00C2\u00A00.9\t\r \u00C2\u00A00.4\t\r \u00C2\u00A00.01\t\r \u00C2\u00A0 \t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Hayden\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Beighton\t\r \u00C2\u00A01977,\t\r \u00C2\u00A0Hayden\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01980\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Asia\t\r \u00C2\u00A0Taiwan\t\r \u00C2\u00A0 2007\t\r \u00C2\u00A0 0.42\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Chen\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Lai\t\r \u00C2\u00A02007\t\r \u00C2\u00A0China\t\r \u00C2\u00A0(Hong\t\r \u00C2\u00A0Kong)\t\r \u00C2\u00A0 1984-\u00C2\u00AD\u00E2\u0080\u00901991\t\r \u00C2\u00A0 0.4\t\r \u00C2\u00A00.4\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Leung\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01992\t\r \u00C2\u00A0India\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 1.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Shiwach\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Lindenbaum\t\r \u00C2\u00A01992\t\r \u00C2\u00A0Japan\t\r \u00C2\u00A0 1997\t\r \u00C2\u00A0 0.7\t\r \u00C2\u00A00.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Adachi\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Nakashima\t\r \u00C2\u00A01999\t\r \u00C2\u00A0Guam\t\r \u00C2\u00A0(Chamorros)\t\r \u00C2\u00A0 1967\t\r \u00C2\u00A0 0.0\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Chen\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A01968\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 0.5\t\r \u00C2\u00A0 1.0\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Australia\t\r \u00C2\u00A0&\t\r \u00C2\u00A0New\t\r \u00C2\u00A0Zealand\t\r \u00C2\u00A0Australia\t\r \u00C2\u00A0 1994\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 27.5\t\r \u00C2\u00A0 Taylor\t\r \u00C2\u00A01994\t\r \u00C2\u00A0New\t\r \u00C2\u00A0South\t\r \u00C2\u00A0Wales\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 6.3\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 McCusker\t\r \u00C2\u00A0et\t\r \u00C2\u00A0al.\t\r \u00C2\u00A02000\t\r \u00C2\u00A0Queensland\t\r \u00C2\u00A0 1976\t\r \u00C2\u00A0 5.8\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Wallace\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Parker\t\r \u00C2\u00A01979\t\r \u00C2\u00A0Tasmania\t\r \u00C2\u00A0 1990\t\r \u00C2\u00A0 12.1\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Pridmore\t\r \u00C2\u00A01990\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0 1963\t\r \u00C2\u00A0 4.6\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Brothers\t\r \u00C2\u00A01964\t\r \u00C2\u00A0New\t\r \u00C2\u00A0Zealand\t\r \u00C2\u00A0 1996\t\r \u00C2\u00A0 5.7\t\r \u00C2\u00A0 -\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Harper\t\r \u00C2\u00A01996\t\r \u00C2\u00A0Regional\t\r \u00C2\u00A0average\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 6.8\t\r \u00C2\u00A0 Not\t\r \u00C2\u00A0calculated\t\r \u00C2\u00A0 27.5\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 97\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A02\t\r \u00C2\u00A0 A\t\r \u00C2\u00A0list\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0field\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0database\t\r \u00C2\u00A0and\t\r \u00C2\u00A0a\t\r \u00C2\u00A0description\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Field\t\r \u00C2\u00A0Name\t\r \u00C2\u00A0 Field\t\r \u00C2\u00A0Description\t\r \u00C2\u00A0 HD\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0Data\t\r \u00C2\u00A0Table\t\r \u00C2\u00A0Personal\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0Unique\t\r \u00C2\u00A0ID\t\r \u00C2\u00A0#\t\r \u00C2\u00A0 A\t\r \u00C2\u00A0number\t\r \u00C2\u00A0unique\t\r \u00C2\u00A0to\t\r \u00C2\u00A0each\t\r \u00C2\u00A0individual\t\r \u00C2\u00A0entered\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0database\t\r \u00C2\u00A0Gender\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Origin\t\r \u00C2\u00A0 Country\t\r \u00C2\u00A0of\t\r \u00C2\u00A0origin\t\r \u00C2\u00A0Ethnicity\t\r \u00C2\u00A0 Field\t\r \u00C2\u00A0options\t\r \u00C2\u00A0following\t\r \u00C2\u00A0the\t\r \u00C2\u00A0EHDN\t\r \u00C2\u00A0guidelines10\t\r \u00C2\u00A0Date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Birth\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Death\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Cause\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Death\t\r \u00C2\u00A0 What\t\r \u00C2\u00A0was\t\r \u00C2\u00A0the\t\r \u00C2\u00A0direct\t\r \u00C2\u00A0physiological\t\r \u00C2\u00A0cause\t\r \u00C2\u00A0of\t\r \u00C2\u00A0death\t\r \u00C2\u00A0Deceased?\t\r \u00C2\u00A0 Yes\t\r \u00C2\u00A0or\t\r \u00C2\u00A0No\t\r \u00C2\u00A0Demographic\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Authority\t\r \u00C2\u00A0Region\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residency\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Vancouver\t\r \u00C2\u00A0Coastal\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Fraser\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Vancouver\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Northern\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Interior\t\r \u00C2\u00A0City,\t\r \u00C2\u00A0Province\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Postal\t\r \u00C2\u00A0Code\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\u00E2\u0080\u009CUrban\u00E2\u0080\u009D\t\r \u00C2\u00A0or\t\r \u00C2\u00A0\u00E2\u0080\u009CRural\u00E2\u0080\u009D\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0 Rural=\t\r \u00C2\u00A0more\t\r \u00C2\u00A0than\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0Urban=\t\r \u00C2\u00A0within\t\r \u00C2\u00A0two\t\r \u00C2\u00A0hours\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0by\t\r \u00C2\u00A0car\t\r \u00C2\u00A0Referring\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0 Name\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0who\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0to\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0genetics\t\r \u00C2\u00A0or\t\r \u00C2\u00A0Vancouver\u00E2\u0080\u0099s\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Referring\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0city\t\r \u00C2\u00A0 City\t\r \u00C2\u00A0of\t\r \u00C2\u00A0referring\t\r \u00C2\u00A0physician\u00E2\u0080\u0099s\t\r \u00C2\u00A0practice\t\r \u00C2\u00A0Initial\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0Location\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0 Reason\t\r \u00C2\u00A0for\t\r \u00C2\u00A0initial\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0visit:\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 HD\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Referred\t\r \u00C2\u00A0for\t\r \u00C2\u00A0PT\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 PT\t\r \u00C2\u00A0+\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Other\t\r \u00C2\u00A0PT/DT?\t\r \u00C2\u00A0 If\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0an\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0test,\t\r \u00C2\u00A0was\t\r \u00C2\u00A0it\t\r \u00C2\u00A0a\t\r \u00C2\u00A0predictive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0or\t\r \u00C2\u00A0a\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0test?\t\r \u00C2\u00A0Family\t\r \u00C2\u00A0History\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD?\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Yes\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 No\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Unknown\t\r \u00C2\u00A0Genetic\t\r \u00C2\u00A0Test\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0Date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0report\t\r \u00C2\u00A0 From\t\r \u00C2\u00A0the\t\r \u00C2\u00A0DNA\t\r \u00C2\u00A0Diagnostic\t\r \u00C2\u00A0lab\t\r \u00C2\u00A0\t\r \u00C2\u00A0Results\t\r \u00C2\u00A0reported\t\r \u00C2\u00A0to\t\r \u00C2\u00A0patient?\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Yes\t\r \u00C2\u00A0\u00E2\u0080\u00A2 No\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Unknown\t\r \u00C2\u00A0Upper\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0size\t\r \u00C2\u00A0 Dictates\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0test\t\r \u00C2\u00A0results\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0\u00E2\u0089\u00A5\t\r \u00C2\u00A036\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0positive\t\r \u00C2\u00A0test\t\r \u00C2\u00A0result\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Status\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0Risk\t\r \u00C2\u00A0Status\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 50%\t\r \u00C2\u00A0AR\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 25%\t\r \u00C2\u00A0AR\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Unknown\t\r \u00C2\u00A0AR\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Affected\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A010\t\r \u00C2\u00A0The\t\r \u00C2\u00A0European\t\r \u00C2\u00A0Huntington\u00E2\u0080\u0099s\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0network\t\r \u00C2\u00A0(EHDN)\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0multi-\u00C2\u00AD\u00E2\u0080\u0090center\t\r \u00C2\u00A0collaboration\t\r \u00C2\u00A0aiming\t\r \u00C2\u00A0to\t\r \u00C2\u00A0obtain\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0and\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0information\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0large\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0interested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0taking\t\r \u00C2\u00A0part\t\r \u00C2\u00A0in\t\r \u00C2\u00A0various\t\r \u00C2\u00A0HD-\u00C2\u00AD\u00E2\u0080\u0090associated\t\r \u00C2\u00A0studies.\t\r \u00C2\u00A0The\t\r \u00C2\u00A0registry\t\r \u00C2\u00A0maintains\t\r \u00C2\u00A0information\t\r \u00C2\u00A0on\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Europe\t\r \u00C2\u00A0and\t\r \u00C2\u00A0is\t\r \u00C2\u00A0the\t\r \u00C2\u00A0largest\t\r \u00C2\u00A0database\t\r \u00C2\u00A0for\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0world\t\r \u00C2\u00A0(EHDN,\t\r \u00C2\u00A02010).\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 98\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Pre\t\r \u00C2\u00A0Manifest\t\r \u00C2\u00A0(100%\t\r \u00C2\u00A0AR)\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Status\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Symptomatic\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0+\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Clinical\t\r \u00C2\u00A0Dx\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 UNKNOWN\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Unaffected\t\r \u00C2\u00A0(spouse/other)\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 At\t\r \u00C2\u00A0Risk\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Onset\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0Motor\t\r \u00C2\u00A0age/year\t\r \u00C2\u00A0of\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0age/year\t\r \u00C2\u00A0at\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0first\t\r \u00C2\u00A0noted\t\r \u00C2\u00A0to\t\r \u00C2\u00A0have\t\r \u00C2\u00A0experienced\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Chorea/dystonia\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Rigidity\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Difficulty\t\r \u00C2\u00A0with\t\r \u00C2\u00A0balance\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Difficulty\t\r \u00C2\u00A0breathing/swallowing\t\r \u00C2\u00A0Age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Diagnosis\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0age/year\t\r \u00C2\u00A0at\t\r \u00C2\u00A0which\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0a\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0as\t\r \u00C2\u00A0having\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0Age\t\r \u00C2\u00A0at\t\r \u00C2\u00A0onset\t\r \u00C2\u00A0source\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Family\t\r \u00C2\u00A0provided\t\r \u00C2\u00A0information\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Referral\t\r \u00C2\u00A0letter\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 MD\t\r \u00C2\u00A0notes\t\r \u00C2\u00A0UHDRS?\t\r \u00C2\u00A0 Has\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0undergone\t\r \u00C2\u00A0a\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0assessment?\t\r \u00C2\u00A0United\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Rating\t\r \u00C2\u00A0Scale:\t\r \u00C2\u00A0Developed\t\r \u00C2\u00A0by\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Study\t\r \u00C2\u00A0Group\t\r \u00C2\u00A0in\t\r \u00C2\u00A01996ii\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090\t\r \u00C2\u00A0Assessment\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0features\t\r \u00C2\u00A0to\t\r \u00C2\u00A0track\t\r \u00C2\u00A0disease\t\r \u00C2\u00A0progression\t\r \u00C2\u00A0on\t\r \u00C2\u00A0a\t\r \u00C2\u00A0standardized\t\r \u00C2\u00A0scale\t\r \u00C2\u00A0First\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0Item\t\r \u00C2\u00A017\t\r \u00C2\u00A0>\t\r \u00C2\u00A02\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Item\t\r \u00C2\u00A017\t\r \u00C2\u00A0=\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0score\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Sum\t\r \u00C2\u00A0of\t\r \u00C2\u00A0a\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0neurological\t\r \u00C2\u00A0symptom\t\r \u00C2\u00A0assessments\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 0=no\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 4=severe\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0Motor\t\r \u00C2\u00A0Score\t\r \u00C2\u00A0 This\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0diagnostic\t\r \u00C2\u00A0confidence\t\r \u00C2\u00A0score\t\r \u00C2\u00A0(0-\u00C2\u00AD\u00E2\u0080\u00904)\t\r \u00C2\u00A0Most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0 Date\t\r \u00C2\u00A0of\t\r \u00C2\u00A0most\t\r \u00C2\u00A0recent\t\r \u00C2\u00A0UHDRS\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0\t\r \u00C2\u00A0Additional\t\r \u00C2\u00A0Information\t\r \u00C2\u00A0Comments\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Any\t\r \u00C2\u00A0additional\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0status/\t\r \u00C2\u00A0diagnosis\t\r \u00C2\u00A0\u00E2\u0080\u00A2 For\t\r \u00C2\u00A0example\t\r \u00C2\u00A0if\t\r \u00C2\u00A0no\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test\t\r \u00C2\u00A0info\t\r \u00C2\u00A0available\t\r \u00C2\u00A0but\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0mentions\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0tested\t\r \u00C2\u00A0in\t\r \u00C2\u00A0another\t\r \u00C2\u00A0province/country\t\r \u00C2\u00A0\t\r \u00C2\u00A0Additional\t\r \u00C2\u00A0comments\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Additional\t\r \u00C2\u00A0comments\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 For\t\r \u00C2\u00A0example\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0is\t\r \u00C2\u00A0a\t\r \u00C2\u00A0phenocopy\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Any\t\r \u00C2\u00A0information\t\r \u00C2\u00A0of\t\r \u00C2\u00A0interest\t\r \u00C2\u00A0that\t\r \u00C2\u00A0does\t\r \u00C2\u00A0not\t\r \u00C2\u00A0fit\t\r \u00C2\u00A0into\t\r \u00C2\u00A0a\t\r \u00C2\u00A0database\t\r \u00C2\u00A0field\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Risk\t\r \u00C2\u00A0Table\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0number\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0family\t\r \u00C2\u00A0number\t\r \u00C2\u00A0\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Members\t\r \u00C2\u00A0of\t\r \u00C2\u00A0the\t\r \u00C2\u00A0same\t\r \u00C2\u00A0family\t\r \u00C2\u00A0share\t\r \u00C2\u00A0this\t\r \u00C2\u00A0common\t\r \u00C2\u00A0number\t\r \u00C2\u00A0Family\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0totals\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0family\t\r \u00C2\u00A0at\t\r \u00C2\u00A0each\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0category\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 BC\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0only\t\r \u00C2\u00A0Total\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0\u00E2\u0080\u0093\t\r \u00C2\u00A0Not\t\r \u00C2\u00A0yet\t\r \u00C2\u00A0in\t\r \u00C2\u00A0database\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 Total\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0living\t\r \u00C2\u00A0individuals\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0family\t\r \u00C2\u00A0\u00E2\u0080\u00A2 Not\t\r \u00C2\u00A0a\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0 \u00E2\u0080\u00A2 BC\t\r \u00C2\u00A0residents\t\r \u00C2\u00A0only\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 99\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A03\t\r \u00C2\u00A0\t\r \u00C2\u00A0 List\t\r \u00C2\u00A0of\t\r \u00C2\u00A0each\t\r \u00C2\u00A0long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0home\t\r \u00C2\u00A0contacted\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 Nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0 City\t\r \u00C2\u00A01\t\r \u00C2\u00A0 Cormorant\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Alert\t\r \u00C2\u00A0Bay\t\r \u00C2\u00A02\t\r \u00C2\u00A0 Lakes\t\r \u00C2\u00A0District\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Burns\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A03\t\r \u00C2\u00A0 Sunshine\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0 Campbell\t\r \u00C2\u00A0River\t\r \u00C2\u00A04\t\r \u00C2\u00A0 McKim\t\r \u00C2\u00A0Cottage\t\r \u00C2\u00A0 Castlegar\t\r \u00C2\u00A05\t\r \u00C2\u00A0 Talarico\t\r \u00C2\u00A0Place\t\r \u00C2\u00A0 Castlegar\t\r \u00C2\u00A06\t\r \u00C2\u00A0 Burquitlam\t\r \u00C2\u00A0Lions\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Chemainus\t\r \u00C2\u00A07\t\r \u00C2\u00A0 Dr.\t\r \u00C2\u00A0F.W.\t\r \u00C2\u00A0Greene\t\r \u00C2\u00A0Memorial\t\r \u00C2\u00A0Home\t\r \u00C2\u00A0 Cranbrook\t\r \u00C2\u00A08\t\r \u00C2\u00A0 Hillcrest\t\r \u00C2\u00A0Extended\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Unit\t\r \u00C2\u00A0 Creston\t\r \u00C2\u00A09\t\r \u00C2\u00A0 Swan\t\r \u00C2\u00A0Valley\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0 Creston\t\r \u00C2\u00A0\t\r \u00C2\u00A010\t\r \u00C2\u00A0 Fort\t\r \u00C2\u00A0Nelson\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Unit\t\r \u00C2\u00A0 Fort\t\r \u00C2\u00A0Nelson\t\r \u00C2\u00A011\t\r \u00C2\u00A0 North\t\r \u00C2\u00A0Peace\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Fort\t\r \u00C2\u00A0St.\t\r \u00C2\u00A0John\t\r \u00C2\u00A012\t\r \u00C2\u00A0 North\t\r \u00C2\u00A0Peace\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Fort\t\r \u00C2\u00A0St.\t\r \u00C2\u00A0John\t\r \u00C2\u00A013\t\r \u00C2\u00A0 Hardy\t\r \u00C2\u00A0View\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0Intermediate\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0 Grand\t\r \u00C2\u00A0Forks\t\r \u00C2\u00A014\t\r \u00C2\u00A0 Sunshine\t\r \u00C2\u00A0Manor\t\r \u00C2\u00A0Extended\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Unit\t\r \u00C2\u00A0 Grand\t\r \u00C2\u00A0Forks\t\r \u00C2\u00A015\t\r \u00C2\u00A0 Granisle\t\r \u00C2\u00A0Community\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Granisle\t\r \u00C2\u00A016\t\r \u00C2\u00A0 Hudson's\t\r \u00C2\u00A0Hope\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Hudson\u00E2\u0080\u0099s\t\r \u00C2\u00A0Hope\t\r \u00C2\u00A017\t\r \u00C2\u00A0 Cottonwoods\t\r \u00C2\u00A0Extended\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Kelowna\t\r \u00C2\u00A018\t\r \u00C2\u00A0 David\t\r \u00C2\u00A0Lloyd-\u00C2\u00AD\u00E2\u0080\u0090Jones\t\r \u00C2\u00A0Home\t\r \u00C2\u00A0 Kelowna\t\r \u00C2\u00A019\t\r \u00C2\u00A0 Lake\t\r \u00C2\u00A0Country\t\r \u00C2\u00A0Manor\t\r \u00C2\u00A0-\u00C2\u00AD\u00E2\u0080\u0090Pleasant\t\r \u00C2\u00A0Meadows\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0 Lake\t\r \u00C2\u00A0Country\t\r \u00C2\u00A020\t\r \u00C2\u00A0 Mackenzie\t\r \u00C2\u00A0and\t\r \u00C2\u00A0District\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0and\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Mackenzie\t\r \u00C2\u00A021\t\r \u00C2\u00A0 McBride\t\r \u00C2\u00A0and\t\r \u00C2\u00A0District\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0 McBride\t\r \u00C2\u00A0\t\r \u00C2\u00A022\t\r \u00C2\u00A0 Pleasant\t\r \u00C2\u00A0View\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Home\t\r \u00C2\u00A0 Mission\t\r \u00C2\u00A0\t\r \u00C2\u00A023\t\r \u00C2\u00A0 Mountain\t\r \u00C2\u00A0Lakes\t\r \u00C2\u00A0 Nelson\t\r \u00C2\u00A024\t\r \u00C2\u00A0 Country\t\r \u00C2\u00A0Squire\t\r \u00C2\u00A0Retirement\t\r \u00C2\u00A0Villa\t\r \u00C2\u00A0 Osoyoos\t\r \u00C2\u00A025\t\r \u00C2\u00A0 Arrowsmith\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0 Parksville\t\r \u00C2\u00A0\t\r \u00C2\u00A026\t\r \u00C2\u00A0 Penticton\t\r \u00C2\u00A0ECU\t\r \u00C2\u00A0 Penticton\t\r \u00C2\u00A027\t\r \u00C2\u00A0 Tsawaayuus\t\r \u00C2\u00A0(Rainbow\t\r \u00C2\u00A0Gardens)\t\r \u00C2\u00A0 Port\t\r \u00C2\u00A0Alberni\t\r \u00C2\u00A028\t\r \u00C2\u00A0 Westhaven\t\r \u00C2\u00A0 Port\t\r \u00C2\u00A0Alberni\t\r \u00C2\u00A029\t\r \u00C2\u00A0 Peace\t\r \u00C2\u00A0River\t\r \u00C2\u00A0Haven\t\r \u00C2\u00A0 Pouce\t\r \u00C2\u00A0Coupe\t\r \u00C2\u00A030\t\r \u00C2\u00A0 Pouce\t\r \u00C2\u00A0Coupe\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Home\t\r \u00C2\u00A0 Pouce\t\r \u00C2\u00A0Coupe\t\r \u00C2\u00A031\t\r \u00C2\u00A0 Evergreen\t\r \u00C2\u00A0Extended\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0 Powell\t\r \u00C2\u00A0River\t\r \u00C2\u00A032\t\r \u00C2\u00A0 Olive\t\r \u00C2\u00A0Devaud\t\r \u00C2\u00A0Residence\t\r \u00C2\u00A0 Powell\t\r \u00C2\u00A0River\t\r \u00C2\u00A033\t\r \u00C2\u00A0 Acropolis\t\r \u00C2\u00A0Manor\t\r \u00C2\u00A0 Prince\t\r \u00C2\u00A0Rupert\t\r \u00C2\u00A034\t\r \u00C2\u00A0 Prince\t\r \u00C2\u00A0Rupert\t\r \u00C2\u00A0Community\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0 Prince\t\r \u00C2\u00A0Rupert\t\r \u00C2\u00A035\t\r \u00C2\u00A0 Ridgewood\t\r \u00C2\u00A0Lodge\t\r \u00C2\u00A0 Princeton\t\r \u00C2\u00A036\t\r \u00C2\u00A0 Eagle\t\r \u00C2\u00A0Park\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Facility\t\r \u00C2\u00A0 Qualicum\t\r \u00C2\u00A0Beach\t\r \u00C2\u00A037\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0Gardens\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Qualicum\t\r \u00C2\u00A0Beach\t\r \u00C2\u00A0 Qualicum\t\r \u00C2\u00A0Beach\t\r \u00C2\u00A038\t\r \u00C2\u00A0 Moberly\t\r \u00C2\u00A0Park\t\r \u00C2\u00A0Manor\t\r \u00C2\u00A0 Revelstoke\t\r \u00C2\u00A039\t\r \u00C2\u00A0 Hilltop\t\r \u00C2\u00A0House\t\r \u00C2\u00A0 Squamish\t\r \u00C2\u00A040\t\r \u00C2\u00A0 Kelly\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Summerland\t\r \u00C2\u00A041\t\r \u00C2\u00A0 Evergreen\t\r \u00C2\u00A0Hamlets\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Surrey\t\r \u00C2\u00A042\t\r \u00C2\u00A0 Rosewood\t\r \u00C2\u00A0 Trail\t\r \u00C2\u00A043\t\r \u00C2\u00A0 Harbour\t\r \u00C2\u00A0House\t\r \u00C2\u00A0 Trail\t\r \u00C2\u00A0\t\r \u00C2\u00A044\t\r \u00C2\u00A0 Tumbler\t\r \u00C2\u00A0Ridge\t\r \u00C2\u00A0Community\t\r \u00C2\u00A0Health\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Tumbler\t\r \u00C2\u00A0Ridge\t\r \u00C2\u00A045\t\r \u00C2\u00A0 Gateby\t\r \u00C2\u00A0Intermediate\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Facility\t\r \u00C2\u00A0 Vernon\t\r \u00C2\u00A046\t\r \u00C2\u00A0 Noric\t\r \u00C2\u00A0House\t\r \u00C2\u00A0 Vernon\t\r \u00C2\u00A047\t\r \u00C2\u00A0 Heritage\t\r \u00C2\u00A0Square\t\r \u00C2\u00A0 Vernon\t\r \u00C2\u00A048\t\r \u00C2\u00A0 Brookhaven\t\r \u00C2\u00A0Extended\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Westbank\t\r \u00C2\u00A049\t\r \u00C2\u00A0 Westside\t\r \u00C2\u00A0Care\t\r \u00C2\u00A0Centre\t\r \u00C2\u00A0 Westbank\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 100\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A04\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0content\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Question\t\r \u00C2\u00A0 number\t\r \u00C2\u00A0 Question\t\r \u00C2\u00A0 1a\t\r \u00C2\u00A0 Do\t\r \u00C2\u00A0you\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0care\t\r \u00C2\u00A0for\t\r \u00C2\u00A0any\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease?\t\r \u00C2\u00A0Y/N\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1b\t\r \u00C2\u00A0 If\t\r \u00C2\u00A0yes,\t\r \u00C2\u00A0how\t\r \u00C2\u00A0many\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0in\t\r \u00C2\u00A0total\t\r \u00C2\u00A0are\t\r \u00C2\u00A0under\t\r \u00C2\u00A0your\t\r \u00C2\u00A0care\t\r \u00C2\u00A0or\t\r \u00C2\u00A0supervision?\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1c\t\r \u00C2\u00A0 How\t\r \u00C2\u00A0many\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0genetically\t\r \u00C2\u00A0confirmed\t\r \u00C2\u00A0to\t\r \u00C2\u00A0carry\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0mutation\t\r \u00C2\u00A0(\u00E2\u0089\u00A536\t\r \u00C2\u00A0CAG\t\r \u00C2\u00A0repeats\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0HTT\t\r \u00C2\u00A0gene)?\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1d\t\r \u00C2\u00A0 How\t\r \u00C2\u00A0many\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0diagnosed\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0solely\t\r \u00C2\u00A0via\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0presentation\t\r \u00C2\u00A0and\t\r \u00C2\u00A0family\t\r \u00C2\u00A0history\t\r \u00C2\u00A0(without\t\r \u00C2\u00A0a\t\r \u00C2\u00A0genetic\t\r \u00C2\u00A0test)?\t\r \u00C2\u00A0\t\r \u00C2\u00A0 2a\t\r \u00C2\u00A0 To\t\r \u00C2\u00A0the\t\r \u00C2\u00A0best\t\r \u00C2\u00A0of\t\r \u00C2\u00A0your\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0do\t\r \u00C2\u00A0any\t\r \u00C2\u00A0of\t\r \u00C2\u00A0these\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0living\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0residing\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0currently\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0clinical\t\r \u00C2\u00A0symptoms\t\r \u00C2\u00A0of\t\r \u00C2\u00A0HD?\t\r \u00C2\u00A0\t\r \u00C2\u00A0 2b\t\r \u00C2\u00A0 If\t\r \u00C2\u00A0yes,\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0best\t\r \u00C2\u00A0of\t\r \u00C2\u00A0your\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0how\t\r \u00C2\u00A0many\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0relatives?\t\r \u00C2\u00A0\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 If\t\r \u00C2\u00A0any\t\r \u00C2\u00A0of\t\r \u00C2\u00A0your\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0and\t\r \u00C2\u00A0or/their\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0live\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0province,\t\r \u00C2\u00A0please\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0both\t\r \u00C2\u00A0in\t\r \u00C2\u00A0BC\t\r \u00C2\u00A0and\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0province\t\r \u00C2\u00A0\t\r \u00C2\u00A0 4a\t\r \u00C2\u00A0 In\t\r \u00C2\u00A0order\t\r \u00C2\u00A0to\t\r \u00C2\u00A0avoid\t\r \u00C2\u00A0double\t\r \u00C2\u00A0counting\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0for\t\r \u00C2\u00A0this\t\r \u00C2\u00A0study,\t\r \u00C2\u00A0it\t\r \u00C2\u00A0is\t\r \u00C2\u00A0important\t\r \u00C2\u00A0to\t\r \u00C2\u00A0know\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0your\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0also\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Clinic\t\r \u00C2\u00A0at\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0department\t\r \u00C2\u00A0at\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0\t\r \u00C2\u00A0 4b\t\r \u00C2\u00A0 To\t\r \u00C2\u00A0the\t\r \u00C2\u00A0best\t\r \u00C2\u00A0of\t\r \u00C2\u00A0your\t\r \u00C2\u00A0knowledge,\t\r \u00C2\u00A0please\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0been\t\r \u00C2\u00A0referred\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0Huntington\t\r \u00C2\u00A0Disease\t\r \u00C2\u00A0medical\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0General\t\r \u00C2\u00A0Hospital\t\r \u00C2\u00A0Medical\t\r \u00C2\u00A0Genetics\t\r \u00C2\u00A0and\t\r \u00C2\u00A0those\t\r \u00C2\u00A0who\t\r \u00C2\u00A0have\t\r \u00C2\u00A0not.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0content\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 101\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A05\t\r \u00C2\u00A0\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0breakdown\t\r \u00C2\u00A0of\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measures\t\r \u00C2\u00A0showing\t\r \u00C2\u00A0the\t\r \u00C2\u00A0number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0last\t\r \u00C2\u00A0updated\t\r \u00C2\u00A0 on\t\r \u00C2\u00A0each\t\r \u00C2\u00A0particular\t\r \u00C2\u00A0year\t\r \u00C2\u00A0and\t\r \u00C2\u00A0the\t\r \u00C2\u00A0corresponding\t\r \u00C2\u00A0certainty\t\r \u00C2\u00A0measure\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Year\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0last\t\r \u00C2\u00A0 updated\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0 from\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0Unknown\t\r \u00C2\u00A0 18\t\r \u00C2\u00A01995\t\r \u00C2\u00A0 1\t\r \u00C2\u00A01996\t\r \u00C2\u00A0 2\t\r \u00C2\u00A01997\t\r \u00C2\u00A0 2\t\r \u00C2\u00A01998\t\r \u00C2\u00A0 3\t\r \u00C2\u00A01999\t\r \u00C2\u00A0 5\t\r \u00C2\u00A02000\t\r \u00C2\u00A0 4\t\r \u00C2\u00A02001\t\r \u00C2\u00A0 4\t\r \u00C2\u00A02002\t\r \u00C2\u00A0 3\t\r \u00C2\u00A02003\t\r \u00C2\u00A0 15\t\r \u00C2\u00A02004\t\r \u00C2\u00A0 7\t\r \u00C2\u00A02005\t\r \u00C2\u00A0 5\t\r \u00C2\u00A02006\t\r \u00C2\u00A0 3\t\r \u00C2\u00A02007\t\r \u00C2\u00A0 8\t\r \u00C2\u00A02008\t\r \u00C2\u00A0 6\t\r \u00C2\u00A02009\t\r \u00C2\u00A0 3\t\r \u00C2\u00A02010\t\r \u00C2\u00A0 8\t\r \u00C2\u00A02011\t\r \u00C2\u00A0 26\t\r \u00C2\u00A02012\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 127\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 102\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A06\t\r \u00C2\u00A0 City\t\r \u00C2\u00A0 Chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0(UBC/VGH)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Pedigree/\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0file\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(patients)\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(Family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Surveys\t\r \u00C2\u00A0 Long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0justification\t\r \u00C2\u00A0 100\t\r \u00C2\u00A0Mile\t\r \u00C2\u00A0House\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Abbotsford\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Aiyansh\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Aldergrove\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Agassiz\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A01\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Armstrong\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A01\t\r \u00C2\u00A0 A\t\r \u00C2\u00A0Ashcroft\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Bella\t\r \u00C2\u00A0Coola\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Berriere\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Blind\t\r \u00C2\u00A0Bay\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Burnaby\t\r \u00C2\u00A0 18\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Burns\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Campbell\t\r \u00C2\u00A0River\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Castlegar\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Chase\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Chemainus\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Chilliwack\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A04\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Christina\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Clearwater\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Cobble\t\r \u00C2\u00A0Hill\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Comox\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Coquitlam\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Courtenay\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Cranbrook\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Crawford\t\r \u00C2\u00A0Bay\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Creston\t\r 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\u00C2\u00A0Kelowna\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Keremeos\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Kimberley\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A01,\t\r \u00C2\u00A01\t\r \u00C2\u00A0 F\t\r \u00C2\u00A0Kitamat\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 103\t\r \u00C2\u00A0 City\t\r \u00C2\u00A0 Chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0(UBC/VGH)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Pedigree/\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0file\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(patients)\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(Family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Surveys\t\r \u00C2\u00A0 Long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0justification\t\r \u00C2\u00A0 Koksilah\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A01\t\r \u00C2\u00A0 G\t\r \u00C2\u00A0Lac\t\r \u00C2\u00A0la\t\r \u00C2\u00A0Hache\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Ladysmith\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0Cowichan\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Langford\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Langley\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lantzville\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lazo\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lillooet\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Logan\t\r \u00C2\u00A0lake\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lumby\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Lytton\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mackenzie\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Madeira\t\r \u00C2\u00A0Park\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Malahat\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Maple\t\r \u00C2\u00A0Ridge\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mara\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Masons\t\r \u00C2\u00A0Landing\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Masset\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mayne\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0McBride\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Meritt\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mill\t\r \u00C2\u00A0Bay\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Mission\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Nakusp\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Nanaimo\t\r \u00C2\u00A0 13\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2,\t\r \u00C2\u00A02,\t\r \u00C2\u00A02,\t\r \u00C2\u00A02,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 H\t\r \u00C2\u00A0Nanoose\t\r \u00C2\u00A0Bay\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Naramata\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Nelson\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0New\t\r \u00C2\u00A0Denver\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0New\t\r \u00C2\u00A0Westminster\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0North\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0 15\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Parksville\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Pemberton\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Pender\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Penticton\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 I\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alberni\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alice\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0(x10)\t\r \u00C2\u00A0 J\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Coquitlam\t\r \u00C2\u00A0 9\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Hardy\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Moody\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Powell\t\r \u00C2\u00A0River\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A01,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 K\t\r \u00C2\u00A0Prince\t\r \u00C2\u00A0George\t\r \u00C2\u00A0 14\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Prince\t\r \u00C2\u00A0Rupert\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A02\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Princeton\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 L\t\r \u00C2\u00A0Pritchard\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Quadra\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Qualicum\t\r \u00C2\u00A0Beach\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Quathiaski\t\r \u00C2\u00A0Cove\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Queen\t\r \u00C2\u00A0Charlotte\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Quesnel\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Richmond\t\r \u00C2\u00A0 10\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Rossland\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Salmon\t\r \u00C2\u00A0Arm\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Saltspring\t\r \u00C2\u00A0Island\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Sayward\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Sechelt\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Shawnigan\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 104\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Note:\t\r \u00C2\u00A0\t\r \u00C2\u00A0Cells\t\r \u00C2\u00A0shaded\t\r \u00C2\u00A0in\t\r \u00C2\u00A0grey\t\r \u00C2\u00A0contain\t\r \u00C2\u00A0numbers\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0who\t\r \u00C2\u00A0are\t\r \u00C2\u00A0cases\t\r \u00C2\u00A0of\t\r \u00C2\u00A0definite\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0and\t\r \u00C2\u00A0have\t\r \u00C2\u00A0not\t\r \u00C2\u00A0been\t\r \u00C2\u00A0included\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0ORS\t\r \u00C2\u00A0assessment\t\r \u00C2\u00A0or\t\r \u00C2\u00A0the\t\r \u00C2\u00A0prevalence\t\r \u00C2\u00A0estimates.\t\r \u00C2\u00A0Numbers\t\r \u00C2\u00A0coloured\t\r \u00C2\u00A0orange\t\r \u00C2\u00A0in\t\r \u00C2\u00A0column\t\r \u00C2\u00A02\t\r \u00C2\u00A0are\t\r \u00C2\u00A0those\t\r \u00C2\u00A011\t\r \u00C2\u00A0out\t\r \u00C2\u00A0of\t\r \u00C2\u00A0127\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0found\t\r \u00C2\u00A0on\t\r \u00C2\u00A0family\t\r \u00C2\u00A0pedigrees\t\r \u00C2\u00A0whose\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0included\t\r \u00C2\u00A0city\t\r \u00C2\u00A0of\t\r \u00C2\u00A0residence\t\r \u00C2\u00A0information\t\r \u00C2\u00A0for\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0in\t\r \u00C2\u00A0question.\t\r \u00C2\u00A0\t\r \u00C2\u00A0For\t\r \u00C2\u00A0the\t\r \u00C2\u00A0remaining\t\r \u00C2\u00A0116\t\r \u00C2\u00A0patients,\t\r \u00C2\u00A0first-\u00C2\u00AD\u00E2\u0080\u0090degree\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\u00E2\u0080\u0099\t\r \u00C2\u00A0resident\t\r \u00C2\u00A0cities\t\r \u00C2\u00A0were\t\r \u00C2\u00A0recorded.\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0justifications:\t\r \u00C2\u00A0\t\r \u00C2\u00A0 A. None\t\r \u00C2\u00A0of\t\r \u00C2\u00A0our\t\r \u00C2\u00A0families\t\r \u00C2\u00A0in\t\r \u00C2\u00A0this\t\r \u00C2\u00A0Armstrong\t\r \u00C2\u00A0have\t\r \u00C2\u00A0an\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0B. Three\t\r \u00C2\u00A0were\t\r \u00C2\u00A0given\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A01\t\r \u00C2\u00A0because\t\r \u00C2\u00A0there\t\r \u00C2\u00A0are\t\r \u00C2\u00A0only\t\r \u00C2\u00A03\t\r \u00C2\u00A0total\t\r \u00C2\u00A0Ferni\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0and\t\r \u00C2\u00A03\t\r \u00C2\u00A0were\t\r \u00C2\u00A0given\t\r \u00C2\u00A0an\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0of\t\r \u00C2\u00A02\t\r \u00C2\u00A0because\t\r \u00C2\u00A0there\t\r \u00C2\u00A0is\t\r \u00C2\u00A0no\t\r \u00C2\u00A0information\t\r \u00C2\u00A0in\t\r \u00C2\u00A0the\t\r \u00C2\u00A0file\t\r \u00C2\u00A0to\t\r \u00C2\u00A0confirm\t\r \u00C2\u00A0with\t\r \u00C2\u00A0the\t\r \u00C2\u00A0physician\t\r \u00C2\u00A0(non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0was\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0referral\t\r \u00C2\u00A0letter)\t\r \u00C2\u00A0C. Physician\t\r \u00C2\u00A0could\t\r \u00C2\u00A0not\t\r \u00C2\u00A0be\t\r \u00C2\u00A0reached\t\r \u00C2\u00A0to\t\r \u00C2\u00A0answer\t\r \u00C2\u00A0pertinent\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0D. No\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0are\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0to\t\r \u00C2\u00A0live\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Invermere\t\r \u00C2\u00A0E. Multiple\t\r \u00C2\u00A0families\t\r \u00C2\u00A0with\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0members\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0live\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Kamloops.\t\r \u00C2\u00A0There\t\r \u00C2\u00A0is\t\r \u00C2\u00A0far\t\r \u00C2\u00A0too\t\r \u00C2\u00A0long\t\r \u00C2\u00A0a\t\r \u00C2\u00A0list\t\r \u00C2\u00A0to\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contact\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0with\t\r \u00C2\u00A0questions.\t\r \u00C2\u00A0F. Patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Kimberley\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0any\t\r \u00C2\u00A0other\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0G. Patients\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Koksilah\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0any\t\r \u00C2\u00A0other\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0H. Physician\t\r \u00C2\u00A0did\t\r \u00C2\u00A0not\t\r \u00C2\u00A0have\t\r \u00C2\u00A0access\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0information\t\r \u00C2\u00A0required\t\r \u00C2\u00A0to\t\r \u00C2\u00A0minimize\t\r \u00C2\u00A0overlap\t\r \u00C2\u00A0I. Nursing\t\r \u00C2\u00A0home\t\r \u00C2\u00A0staff\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0able\t\r \u00C2\u00A0to\t\r \u00C2\u00A0provide\t\r \u00C2\u00A0information\t\r \u00C2\u00A0on\t\r \u00C2\u00A0whether\t\r \u00C2\u00A0this\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0has\t\r \u00C2\u00A0been\t\r \u00C2\u00A0to\t\r \u00C2\u00A0the\t\r \u00C2\u00A0UBC\t\r \u00C2\u00A0HD\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0or\t\r \u00C2\u00A0VGH\t\r \u00C2\u00A0nor\t\r \u00C2\u00A0did\t\r \u00C2\u00A0they\t\r \u00C2\u00A0have\t\r \u00C2\u00A0information\t\r \u00C2\u00A0regarding\t\r \u00C2\u00A0the\t\r \u00C2\u00A0patient\u00E2\u0080\u0099s\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0 City\t\r \u00C2\u00A0 Chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0(UBC/VGH)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Pedigree/\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0file\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(patients)\t\r \u00C2\u00A0 Physician\t\r \u00C2\u00A0responses\t\r \u00C2\u00A0(Family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0with\t\r \u00C2\u00A0HD)\t\r \u00C2\u00A0 Family\t\r \u00C2\u00A0Surveys\t\r \u00C2\u00A0 Long-\u00C2\u00AD\u00E2\u0080\u0090term\t\r \u00C2\u00A0care\t\r \u00C2\u00A0homes\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0scores\t\r \u00C2\u00A0 Overlap\t\r \u00C2\u00A0risk\t\r \u00C2\u00A0score\t\r \u00C2\u00A0justification\t\r \u00C2\u00A0 Slocan\t\r \u00C2\u00A0Park\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Smithers\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Sooke\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Southbank\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Sparwood\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A03\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Squamish\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Stewart\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Summerland\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Surrey\t\r \u00C2\u00A0 42\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 14\t\r \u00C2\u00A0 2,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 M\t\r \u00C2\u00A0Terrace\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Trail\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Tsawwassen\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1,\t\r \u00C2\u00A01,\t\r \u00C2\u00A01,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 N\t\r \u00C2\u00A0Ucluelet\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Unknown\t\r \u00C2\u00A0\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Valemount\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0 76\t\r \u00C2\u00A0 15,\t\r \u00C2\u00A01\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Vanderhoof\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 O\t\r \u00C2\u00A0Vernon\t\r \u00C2\u00A0 7\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Victoria\t\r \u00C2\u00A0 67\t\r \u00C2\u00A0 10,\t\r \u00C2\u00A03\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2,\t\r \u00C2\u00A02,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 P\t\r \u00C2\u00A0Watson\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0West\t\r \u00C2\u00A0Vancouver\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Westbank\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 2,\t\r \u00C2\u00A02\t\r \u00C2\u00A0 Q\t\r \u00C2\u00A0Whistler\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 8\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0Whiterock\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0Williams\t\r \u00C2\u00A0Lake\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 2,2\t\r \u00C2\u00A0 R\t\r \u00C2\u00A0Winfield\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 \t\r \u00C2\u00A0 105\t\r \u00C2\u00A0 J. These\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0have\t\r \u00C2\u00A0no\t\r \u00C2\u00A0known\t\r \u00C2\u00A0family\t\r \u00C2\u00A0members\t\r \u00C2\u00A0and\t\r \u00C2\u00A0no\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0or\t\r \u00C2\u00A0families\t\r \u00C2\u00A0ascertained\t\r \u00C2\u00A0from\t\r \u00C2\u00A0other\t\r \u00C2\u00A0sources\t\r \u00C2\u00A0live\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Port\t\r \u00C2\u00A0Alice\t\r \u00C2\u00A0K. No\t\r \u00C2\u00A0families\t\r \u00C2\u00A0from\t\r \u00C2\u00A0the\t\r \u00C2\u00A0chart\t\r \u00C2\u00A0review\t\r \u00C2\u00A0have\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0members\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Powell\t\r \u00C2\u00A0River.\t\r \u00C2\u00A0L. Cannot\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contact\t\r \u00C2\u00A0family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0M. 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The\t\r \u00C2\u00A0one\t\r \u00C2\u00A0clinic\t\r \u00C2\u00A0family\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Tsawwassen\t\r \u00C2\u00A0does\t\r \u00C2\u00A0not\t\r \u00C2\u00A0have\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0family\t\r \u00C2\u00A0member,\t\r \u00C2\u00A0and\t\r \u00C2\u00A0only\t\r \u00C2\u00A0has\t\r \u00C2\u00A01\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0from\t\r \u00C2\u00A0Tsawwassen\t\r \u00C2\u00A0is\t\r \u00C2\u00A0recorded\t\r \u00C2\u00A0as\t\r \u00C2\u00A0affected.\t\r \u00C2\u00A0Family\t\r \u00C2\u00A0survey\t\r \u00C2\u00A0responders\t\r \u00C2\u00A0cannot\t\r \u00C2\u00A0be\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contacted\t\r \u00C2\u00A0O. Only\t\r \u00C2\u00A0family\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Vanderhoof\t\r \u00C2\u00A0has\t\r \u00C2\u00A0no\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0not\t\r \u00C2\u00A0in\t\r \u00C2\u00A0database\t\r \u00C2\u00A0members\t\r \u00C2\u00A0and\t\r \u00C2\u00A0saw\t\r \u00C2\u00A0a\t\r \u00C2\u00A0different\t\r \u00C2\u00A0doctor.\t\r \u00C2\u00A0P. Multiple\t\r \u00C2\u00A0families\t\r \u00C2\u00A0with\t\r \u00C2\u00A0non-\u00C2\u00AD\u00E2\u0080\u0090clinic\t\r \u00C2\u00A0patient\t\r \u00C2\u00A0affected\t\r \u00C2\u00A0members\t\r \u00C2\u00A0on\t\r \u00C2\u00A0their\t\r \u00C2\u00A0pedigree\t\r \u00C2\u00A0live\t\r \u00C2\u00A0in\t\r \u00C2\u00A0Victoria.\t\r \u00C2\u00A0There\t\r \u00C2\u00A0is\t\r \u00C2\u00A0far\t\r \u00C2\u00A0too\t\r \u00C2\u00A0long\t\r \u00C2\u00A0a\t\r \u00C2\u00A0list\t\r \u00C2\u00A0to\t\r \u00C2\u00A0re-\u00C2\u00AD\u00E2\u0080\u0090contact\t\r \u00C2\u00A0physicians\t\r \u00C2\u00A0with\t\r \u00C2\u00A0questions.\t\r \u00C2\u00A0Q. 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Physicians\t\r \u00C2\u00A0were\t\r \u00C2\u00A0not\t\r \u00C2\u00A0available\t\r \u00C2\u00A0to\t\r \u00C2\u00A0answer\t\r \u00C2\u00A0the\t\r \u00C2\u00A0pertinent\t\r \u00C2\u00A0questions\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 \t\r \u00C2\u00A0 106\t\r \u00C2\u00A0 Appendix\t\r \u00C2\u00A07\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Ethnicity\t\r \u00C2\u00A0 Country\t\r \u00C2\u00A0of\t\r \u00C2\u00A0orgin\t\r \u00C2\u00A0 Number\t\r \u00C2\u00A0of\t\r \u00C2\u00A0patients\t\r \u00C2\u00A0 Caucasian\t\r \u00C2\u00A0 Acadian/Cajun\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Austria\t\r \u00C2\u00A0 4\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Belgium\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Great\t\r \u00C2\u00A0Britain\t\r \u00C2\u00A0 154\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Hungary\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Iceland\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Ireland\t\r \u00C2\u00A0 30\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Canada\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Denmark\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Poland\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Norway\t\r \u00C2\u00A0 13\t\r \u00C2\u00A0\t\r \u00C2\u00A0 The\t\r \u00C2\u00A0Netherlands\t\r \u00C2\u00A0 16\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Latvia\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Italy\t\r \u00C2\u00A0 5\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Israel\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Germany\t\r \u00C2\u00A0 26\t\r \u00C2\u00A0\t\r \u00C2\u00A0 France\t\r \u00C2\u00A0 6\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Finland\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Russia\t\r \u00C2\u00A0 17\t\r \u00C2\u00A0\t\r \u00C2\u00A0 South\t\r \u00C2\u00A0Africa\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Sweden\t\r \u00C2\u00A0 11\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Ukraine\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Slovania\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Unknown\t\r \u00C2\u00A0 166\t\r \u00C2\u00A0 African-\u00C2\u00AD\u00E2\u0080\u0090North\t\r \u00C2\u00A0 Tanzania\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 American-\u00C2\u00AD\u00E2\u0080\u0090Latin\t\r \u00C2\u00A0 Mexico\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 Asian-\u00C2\u00AD\u00E2\u0080\u0090East\t\r \u00C2\u00A0 Philippines\t\r \u00C2\u00A0\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Hong\t\r \u00C2\u00A0Kong\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Unknown\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0 Asian-\u00C2\u00AD\u00E2\u0080\u0090West\t\r \u00C2\u00A0 Pakistan\t\r \u00C2\u00A0 3\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Afghanistan\t\r \u00C2\u00A0\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 India\t\r \u00C2\u00A0 8\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Iran\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Iraq\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0 Unknown\t\r \u00C2\u00A0 2\t\r \u00C2\u00A0 Other\t\r \u00C2\u00A0 Ontario\t\r \u00C2\u00A0Aboriginal\t\r \u00C2\u00A0 1\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0\t\r \u00C2\u00A0"@en . "Thesis/Dissertation"@en . "2012-11"@en . "10.14288/1.0073226"@en . "eng"@en . "Medical Genetics"@en . "Vancouver : University of British Columbia Library"@en . "University of British Columbia"@en . "Attribution-NonCommercial-NoDerivatives 4.0 International"@en . "http://creativecommons.org/licenses/by-nc-nd/4.0/"@en . "Graduate"@en . "The epidemiology of Huntington disease in British Columbia"@en . "Text"@en . "http://hdl.handle.net/2429/43319"@en .