{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T05:26:40Z","timestamp":1771046800664,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2012,10,3]],"date-time":"2012-10-03T00:00:00Z","timestamp":1349222400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Disorders of collagen are associated with a mild bleeding tendency because of the potential abnormal interaction of collagen, von Willebrand factor (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>) and platelets required during primary haemostasis and due to generalized soft tissue fragility. Abnormal collagen may contribute to bleeding in existing mucocutaneous bleeding disorders, but the prevalence in this setting is unknown. Generalized symptomatic joint hypermobility (<jats:styled-content style=\"fixed-case\">SJH<\/jats:styled-content>) is common in collagen disorders and may be objectively measured. To assess the association between symptomatic joint hypermobility and mucocutaneous bleeding disorders, we performed a case\u2013control study in which case subjects were 55 consecutive individuals who had visited our bleeding disorder clinic with a diagnosis of von Willebrand disease, low von Willebrand factor levels, mild platelet function disorder or undefined bleeding disorder. Controls were 50 subjects without a bleeding disorder, and were age and gender matched to the cases. All subjects were assessed with: (i) Beighton score for joint hypermobility, (ii) revised Brighton criteria, (iii) Condensed <jats:styled-content style=\"fixed-case\">MCMDM<\/jats:styled-content>1\u2010<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> bleeding questionnaire, and (iv) haemostasis laboratory studies. The prevalence of <jats:styled-content style=\"fixed-case\">SJH<\/jats:styled-content>\/suspected collagen disorder in the bleeding disorder clinic was 24% (13\/55) compared with 2% (1\/50) in the control population (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 15, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 2\u2013121). Seventy\u2010seven per cent of bleeding disorder clinic <jats:styled-content style=\"fixed-case\">SJH<\/jats:styled-content> subjects (10\/13) had a prior personal or family history of Ehlers\u2010Danlos, Benign Joint Hypermobility Syndrome or Osteogenesis Imperfecta (<jats:styled-content style=\"fixed-case\">OI<\/jats:styled-content>). Symptomatic joint hypermobility was associated with increased odds of an underlying mucocutaneous bleeding disorder. These findings suggest that a collagen disorder is common and often unrecognized in the bleeding disorder clinic as a potential contributor to the bleeding symptoms.<\/jats:p>","DOI":"10.1111\/hae.12020","type":"journal-article","created":{"date-parts":[[2012,10,3]],"date-time":"2012-10-03T07:23:06Z","timestamp":1349248986000},"page":"246-250","source":"Crossref","is-referenced-by-count":29,"title":["Suspected collagen disorders in the bleeding disorder clinic: A case\u2013control study"],"prefix":"10.1111","volume":"19","author":[{"given":"S. C.","family":"Jackson","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine St. Paul's Hospital, University of British Columbia Vancouver British Columbia Canada"},{"name":"Division of Hematology and Hematologic Malignancies, Department of Medicine University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Odiaman","sequence":"additional","affiliation":[{"name":"Division of Hematology and Hematologic Malignancies, Department of Medicine University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. T.","family":"Card","sequence":"additional","affiliation":[{"name":"Division of Hematology and Hematologic Malignancies, Department of Medicine University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. G.","family":"van der Bom","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology Leiden University Medical Center Leiden the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u2010C.","family":"Poon","sequence":"additional","affiliation":[{"name":"Division of Hematology and Hematologic Malignancies, Department of Medicine University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,10,3]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2004.05220.x"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2004.00665.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1991.tb06063.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.10816"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0028-1104544"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/BOR.0b013e3282f31790"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03182.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1302\/0301-620X.51B3.444"},{"key":"e_1_2_6_10_1","first-page":"1777","article-title":"The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS)","volume":"27","author":"Grahame R","year":"2000","journal-title":"J Rheumatol"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kem290"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2007.12.005"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-006-0101-1"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V88.5.1525.1525"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2009.06.001"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07446.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12020","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12020","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T06:23:35Z","timestamp":1698647015000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12020"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,10,3]]},"references-count":16,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1111\/hae.12020"],"URL":"https:\/\/doi.org\/10.1111\/hae.12020","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,10,3]]}}}