{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:51:40Z","timestamp":1787014300383,"version":"build-2736575974"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,3,17]],"date-time":"2017-03-17T00:00:00Z","timestamp":1489708800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2017,5]]},"abstract":"\n Introduction<\/jats:title>\n \n von Willebrand disease (\n VWD<\/jats:styled-content>\n ) is the most common inherited bleeding disorder. In\n VWD<\/jats:styled-content>\n patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in\n VWD<\/jats:styled-content>\n patients.\n <\/jats:p>\n <\/jats:sec>\n \n Aim<\/jats:title>\n \n To investigate whether plasminogen activator inhibitor\u20101 (\n PAI<\/jats:styled-content>\n \u20101) level influences the variation in bleeding tendency in\n VWD<\/jats:styled-content>\n patients.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n PAI<\/jats:styled-content>\n \u20101 antigen levels were measured in the plasma of 633 patients with moderate or severe\n VWD<\/jats:styled-content>\n who participated in the \u2018Willebrand in the Netherlands\u2019 (WiN) study, a nationwide multicentre cross\u2010sectional study. Bleeding severity was assessed using the Tosetto bleeding score.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n PAI<\/jats:styled-content>\n \u20101 levels increased with age (Spearman's rho: 0.225,\n P<\/jats:italic>\n \u00a0<\u00a00.001) and were higher in men (23 [\n IQR<\/jats:styled-content>\n 12\u201360] vs. 20 [\n IQR<\/jats:styled-content>\n 10\u201344]\u00a0ng\u00a0mL\n \u22121<\/jats:sup>\n in women,\n P<\/jats:italic>\n \u00a0=\u00a00.039), whereas the bleeding score was higher in women (11 [\n IQR<\/jats:styled-content>\n 7\u201317] vs. 9 [\n IQR<\/jats:styled-content>\n 5\u201314] ng\u00a0mL\n \u22121<\/jats:sup>\n in men,\n P<\/jats:italic>\n \u00a0=\u00a00.002). After adjustment for age and sex by stratification,\n PAI<\/jats:styled-content>\n \u20101 level and bleeding score were negatively correlated (Spearman's rho: \u22120.170,\n P<\/jats:italic>\n \u00a0=\u00a00.017) in the group of 196 young (age\u00a0\u2264\u00a045\u00a0year) female\n VWD<\/jats:styled-content>\n patients, accounting for 31% of our study population.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n In young female\n VWD<\/jats:styled-content>\n patients, we observed that low\n PAI<\/jats:styled-content>\n \u20101 levels were associated with a higher bleeding score, which may partly explain the observed variability in bleeding phenotype in\n VWD<\/jats:styled-content>\n patients.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/hae.13206","type":"journal-article","created":{"date-parts":[[2017,3,17]],"date-time":"2017-03-17T09:53:27Z","timestamp":1489744407000},"page":"437-443","source":"Crossref","is-referenced-by-count":8,"title":["Plasma levels of plasminogen activator inhibitor\u20101 and bleeding phenotype in patients with von Willebrand disease"],"prefix":"10.1111","volume":"23","author":[{"given":"S.","family":"Abdul","sequence":"first","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Boender","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. J. M. C.","family":"Malfliet","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Eikenboom","sequence":"additional","affiliation":[{"name":"Department of Thrombosis and Hemostasis Leiden University Medical Center Leiden The Netherlands"},{"name":"Einthoven Laboratory for Experimental Vascular Medicine Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Fijn van Draat","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Emma Children's Hospital AMC Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. P.","family":"Mauser\u2010Bunschoten","sequence":"additional","affiliation":[{"name":"Van Creveldkliniek department of benign Hematology University Medical Center Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Meijer","sequence":"additional","affiliation":[{"name":"Hematology University Medical Center Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"de Meris","sequence":"additional","affiliation":[{"name":"Netherlands Hemophilia Society Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B. A. P.","family":"Laros\u2010van Gorkom","sequence":"additional","affiliation":[{"name":"Hematology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. G.","family":"van der Bom","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology Leiden University Medical Center Leiden The Netherlands"},{"name":"Jon J Van Rood Center for Clinical Transfusion Medicine, Sanquin Research Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. W. G.","family":"Leebeek","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. C.","family":"Rijken","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2035-4485","authenticated-orcid":false,"given":"S.","family":"Uitte de Willige","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus University Medical Center Rotterdam Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"WIN study group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,3,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-06-498303"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-06-528406"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02005.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613992"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-04-0244"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24195"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-01-0349"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14255"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03220.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12935"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-09-670117"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2012.06.023"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01834.x"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01709.x"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02645.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00991.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.11.1.183"},{"key":"e_1_2_8_21_1","first-page":"117","volume-title":"Progress in Fibrinolysis VII","author":"Kluft C","year":"1985"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(86)90328-2"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1642781"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-02-267740"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13206","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.13206","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T12:32:49Z","timestamp":1695472369000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.13206"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,3,17]]},"references-count":23,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017,5]]}},"alternative-id":["10.1111\/hae.13206"],"URL":"https:\/\/doi.org\/10.1111\/hae.13206","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,3,17]]}}}