{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T19:03:04Z","timestamp":1790708584383,"version":"4.1.0"},"reference-count":13,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T00:00:00Z","timestamp":1778544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T00:00:00Z","timestamp":1778544000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["09835303"],"award-info":[{"award-number":["09835303"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["10801776"],"award-info":[{"award-number":["10801776"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["11948954"],"award-info":[{"award-number":["11948954"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["20316866"],"award-info":[{"award-number":["20316866"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["23810379"],"award-info":[{"award-number":["23810379"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["18058983"],"award-info":[{"award-number":["18058983"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["21445007"],"award-info":[{"award-number":["21445007"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["24015773"],"award-info":[{"award-number":["24015773"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Vascular Ehlers\u2013Danlos syndrome (vEDS) is a hereditary connective tissue disorder caused by heterozygous pathogenic variants in\n                    <jats:italic>COL3A1<\/jats:italic>\n                    . European studies have shown that celiprolol may reduce the risk of life\u2010threatening vascular events, but outcomes in non\u2010European populations and the therapy's psychological impact remain unclear. We conducted a retrospective cohort study of individuals aged \u2265\u200920\u2009years with genetically confirmed vEDS in a single referral center in Japan between 2000 and 2024. Clinical, molecular, and treatment data were obtained from medical records, and semi\u2010structured interviews were conducted with a subset of participants to assess patient experiences. Twenty\u2010six patients were included. The mean age at celiprolol initiation was 36.9\u2009years, with a mean follow\u2010up of 96.6\u2009months. All patients received celiprolol, and 61.5% reached the target dose (400\u2009mg\/day). One patient died of hepatic artery rupture; vascular events occurred in 11, while 14 remained event\u2010free. No significant associations were observed between vascular event occurrence and genotype or celiprolol dose. Interviews with 11 patients revealed that celiprolol use provided emotional reassurance and promoted a more proactive approach to disease management. Celiprolol therapy may reduce fatal vascular events in vEDS and have a positive psychological impact, though nonfatal vascular complications remain frequent.\n                  <\/jats:p>","DOI":"10.1002\/ajmg.a.70178","type":"journal-article","created":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T05:36:06Z","timestamp":1778650566000},"page":"2241-2249","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Outcomes and Patient Experiences With Celiprolol Therapy in Vascular Ehlers\u2013Danlos Syndrome: The First Non\u2010European Cohort"],"prefix":"10.1002","volume":"200","author":[{"given":"Megumi","family":"Furuhata\u2010Yoshimura","sequence":"first","affiliation":[{"name":"Department of Medical Genetics Shinshu University School of Medicine  Matsumoto Japan"},{"name":"Department of Pediatrics Aizawa Hospital, Advanced Care Center  Matsumoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomomi","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Shinshu University School of Medicine  Matsumoto Japan"},{"name":"Center for Medical Genetics Shinshu University Hospital  Matsumoto Japan"},{"name":"Division of Clinical Sequencing Shinshu University School of Medicine  Matsumoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8344-7507","authenticated-orcid":false,"given":"Tomoki","family":"Kosho","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Shinshu University School of Medicine  Matsumoto Japan"},{"name":"Center for Medical Genetics Shinshu University Hospital  Matsumoto Japan"},{"name":"Division of Clinical Sequencing Shinshu University School of Medicine  Matsumoto Japan"},{"name":"Research Center for Supports to Advanced Science Shinshu University  Matsumoto Japan"},{"name":"BioBank Shinshu Shinshu University Hospital  Matsumoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejvs.2020.10.020"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000121741.50315.C2"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/1358863X231215330"},{"key":"e_1_2_11_5_1","first-page":"1993","volume-title":"GeneReviews\u00ae [Internet]","author":"Byers P. 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