{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T11:45:49Z","timestamp":1791805549821,"version":"4.3.4"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T00:00:00Z","timestamp":1783296000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T00:00:00Z","timestamp":1783296000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder associated with substantial morbidity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The aim of this study was to estimate mortality in a country without centralized national care for HHT patients. Moreover, we identified causes of death and estimated the prevalence of HHT in Sweden.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      The Swedish National Patient Register (NPR) was used to identify individuals with HHT using the International Classification of Diseases (ICD) code I78.0. Cases included all individuals with this code between January 1, 2001 and December 31, 2003 (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a0393). Five age\u2010 and gender\u2010matched controls from the general population were selected by Statistics Sweden (SCB) (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a01965). Cases and controls were followed for survival until 2018 using the Swedish National Cause of Death Register (DORS). To calculate prevalence, we identified all individuals with the ICD code I78.0 in the NPR over 12 years, from January 1, 2007 to December 30, 2018.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Life expectancy for cases with HHT after age 30 was estimated at 73.0 (68.0\u201377.2) compared to 80.5 (79.1\u201382.0) years for controls. Ischemic heart diseases, diseases of arteries, arterioles, capillaries, and liver diseases were significantly more common causes of death among HHT patients. The prevalence of HHT was estimated at 9.3 per 100,000 over the 12 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>HHT in Sweden is associated with markedly shorter expected survival compared to the general population, and known complications of HHT are among the most common causes of death. The estimated prevalence of HHT in Sweden was relatively low, possibly due to underdiagnosis of those with milder symptoms.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/joim.70134","type":"journal-article","created":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T04:29:14Z","timestamp":1783398554000},"page":"480-488","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Expected survival is decreased in hereditary hemorrhagic telangiectasia: Results from a population\u2010based registry study"],"prefix":"10.1111","volume":"300","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-8788-4127","authenticated-orcid":false,"given":"Cecilia Ahlstr\u00f6m","family":"Emanuelsson","sequence":"first","affiliation":[{"name":"Department of Clinical Science Department of Ear, Nose and Throat Diseases Skane University Hospital  Lund 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