{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T11:45:35Z","timestamp":1791805535537,"version":"4.3.4"},"reference-count":27,"publisher":"Cambridge University Press (CUP)","issue":"1","license":[{"start":{"date-parts":[[2018,12,6]],"date-time":"2018-12-06T00:00:00Z","timestamp":1544054400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. J. Neurol. Sci."],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S0317167118003608_abs1\" sec-type=\"general\"><jats:title>Background<\/jats:title><jats:p>Hereditary hemorrhagic telangiectasia (HHT) is a disease of abnormal vasculature where patients are predisposed to strokes of multiple etiologies. We assessed yearly stroke incidence among Albertans with HHT and compared with the general population. Given the tendency for stroke in HHT patients, we expected HHT patients to have higher stroke incidence, in particular at younger ages.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167118003608_abs2\" sec-type=\"methods\"><jats:title>Methods<\/jats:title><jats:p>Population-based administrative health data on inpatient and ambulatory admissions were extracted over a 16-year period using International Classification of Diseases (ICD)-9 and ICD-10, Canada codes. We analyzed overall occurrence of strokes in Alberta by age, gender, stroke subtype, and diagnosis of HHT.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167118003608_abs3\" sec-type=\"results\"><jats:title>Results<\/jats:title><jats:p>The age-standardized incidence rate of stroke in HHT was 450 per 100,000 compared with 260 per 100,000 in the general population with a rate ratio of 1.73 (95% confidence interval (CI) [1.046-2.842]). This study found a higher HHT prevalence in Alberta (1 in 3800) compared to the world average of 1 in 5000. Women were also more likely to be diagnosed with HHT, with a 3.25:1 female gender preponderance in the yearly incidence.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167118003608_abs4\" sec-type=\"general\"><jats:title>Interpretation<\/jats:title><jats:p>This study not only shows that HHT patients are at higher risk of having a stroke but also quantifies that risk using an age-adjusted metric in Alberta. This province has a higher than expected disease burden of HHT, with the majority of cases affecting women. Our study found that acute ischemic strokes and transient ischemic attacks are far more common than hemorrhage in HHT. As HHT is a rare, multi-system, chronic disease, these patients should be referred to an HHT Centre of Excellence.<\/jats:p><\/jats:sec>","DOI":"10.1017\/cjn.2018.360","type":"journal-article","created":{"date-parts":[[2018,12,6]],"date-time":"2018-12-06T07:58:43Z","timestamp":1544083123000},"page":"44-50","source":"Crossref","is-referenced-by-count":23,"title":["Links Between Strokes and Hereditary Hemorrhagic Telangiectasia: A Population-Based Study"],"prefix":"10.1017","volume":"46","author":[{"given":"Farah N.","family":"Chowdhury","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Sanjaya","family":"Chandrarathne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristopher D.","family":"Masilamani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer T. N.","family":"LaBranche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaun","family":"Malo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence W.","family":"Svenson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Jeerakathil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilini P.","family":"Vethanayagam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2018,12,6]]},"reference":[{"key":"S0317167118003608_ref27","unstructured":"International Classification of Diseases (ICD). World Health Organization. http:\/\/www.who.int\/classificationsicd\/en\/. Accessed December 8, 2014."},{"key":"S0317167118003608_ref12","author":"Jeerakathil","year":"2012"},{"key":"S0317167118003608_ref6","unstructured":"National Institute of Health. National Institute of Neurological Disorders and Stroke. Arteriovenous malformations and other vascular lesions of the central nervous system fact sheet. Available at: http:\/\/www.ninds.nih.gov\/disorders\/avms\/detail_avms.htm. 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