{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:54:42Z","timestamp":1790729682563,"version":"4.1.0"},"reference-count":19,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,11,21]],"date-time":"2016-11-21T00:00:00Z","timestamp":1479686400000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100004326","name":"Bayer","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004326","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2016,11]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use of bleeding assessment tools in this population are lacking.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>Our objective was to validate the <jats:styled-content style=\"fixed-case\">ISTH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">BAT<\/jats:styled-content> in haemophilia carriers to describe bleeding symptoms and allow for comparisons with factor levels and other patient groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a prospective, observational, cross\u2010sectional study performed by members of Global Emerging <jats:styled-content style=\"fixed-case\">HE<\/jats:styled-content>mostasis Panel\u00a0(<jats:styled-content style=\"fixed-case\">GEHEP<\/jats:styled-content>). Unselected consecutive haemophilia carriers were recruited and a <jats:styled-content style=\"fixed-case\">CRF<\/jats:styled-content> and the <jats:styled-content style=\"fixed-case\">ISTH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">BAT<\/jats:styled-content> were completed by study personnel.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 168 haemophilia carriers were enrolled: 155 haemophilia A and 13 haemophilia B. The mean age was 40\u00a0years (range: 20\u201382). Carriers had higher mean bleeding scores (<jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content>) compared with age\u2010matched controls (<jats:italic>n<\/jats:italic>\u00a0=\u00a046; 5.7 vs. 1.43; <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.0001) and Type 3 <jats:styled-content style=\"fixed-case\">VWD OC<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a032; 3.0; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.009), but lower <jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> compared with women with Type 1 <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a083; 8.7; <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.0001). Fifteen carriers reported haemarthrosis, and of those six had normal <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">FIX<\/jats:styled-content> levels. There was a significant but weak negative correlation between <jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> and factor level (Spearman's <jats:italic>r<\/jats:italic><jats:sup>2<\/jats:sup>\u00a0=\u00a0\u22120.36, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our results show that haemophilia carriers experience abnormal bleeding, including haemarthrosis. Overall, <jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> in women with Type 1 <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>\u00a0&gt;\u00a0haemophilia carriers\u00a0&gt;\u00a0Type 3 <jats:styled-content style=\"fixed-case\">VWD OC<\/jats:styled-content>\u00a0&gt;\u00a0controls. Understanding the performance of the <jats:styled-content style=\"fixed-case\">ISTH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">BAT<\/jats:styled-content> in this population is a critical step in future research aimed at investigating the underlying pathophysiology of abnormal bleeding, with the ultimate goal of optimizing treatment.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13089","type":"journal-article","created":{"date-parts":[[2016,11,21]],"date-time":"2016-11-21T08:55:38Z","timestamp":1479718538000},"page":"912-918","source":"Crossref","is-referenced-by-count":98,"title":["Evaluation of the utility of the <scp>ISTH<\/scp>\u2010<scp>BAT<\/scp> in haemophilia carriers: a multinational study"],"prefix":"10.1111","volume":"22","author":[{"given":"P. D.","family":"James","sequence":"first","affiliation":[{"name":"Queen's University Kingston ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Mahlangu","sequence":"additional","affiliation":[{"name":"Faculty of Health Sciences University of the Witwatersrand Johannesburg South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Bidlingmaier","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Children's University Hospital Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. E.","family":"Mingot\u2010Castellano","sequence":"additional","affiliation":[{"name":"Regional University Hospital of Malaga Malaga Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Chitlur","sequence":"additional","affiliation":[{"name":"Children's Hospital of Michigan Detroit MI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. F.","family":"Fogarty","sequence":"additional","affiliation":[{"name":"Perelman School of Medicine University of Pennsylvania Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Cuker","sequence":"additional","affiliation":[{"name":"Perelman School of Medicine University of Pennsylvania Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. E.","family":"Mancuso","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca\u2019 Granda, Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. A.","family":"Holme","sequence":"additional","affiliation":[{"name":"Department of Hematology and Institute of Clinical Medicine University Hospital University of Oslo Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Grabell","sequence":"additional","affiliation":[{"name":"Queen's University Kingston ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Satkunam","sequence":"additional","affiliation":[{"name":"Queen's University Kingston ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. M.","family":"Hopman","sequence":"additional","affiliation":[{"name":"Queen's University Kingston ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Mathew","sequence":"additional","affiliation":[{"name":"Bayer Health Care Whippany NJ USA"},{"name":"University of New Mexico Albuquerque NM USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Global Emerging HEmostasis Experts Panel\u00a0(GEHEP)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,11,21]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1225756"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1615621"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02909.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)61139-2"},{"key":"e_1_2_8_6_1","first-page":"482","article-title":"The phenotypic range of hemophilia A carriers","volume":"28","author":"Graham JB","year":"1976","journal-title":"Am J Hum Genet"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-09-3879"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02426.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02108.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12364"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13423"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000083"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12535"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23754"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000022"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03975.x"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12503"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12928"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12747"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12130"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13089","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13089","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.13089","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T20:27:01Z","timestamp":1695500821000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.13089"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,11]]},"references-count":19,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2016,11]]}},"alternative-id":["10.1111\/hae.13089"],"URL":"https:\/\/doi.org\/10.1111\/hae.13089","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,11]]}}}