{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T13:25:20Z","timestamp":1772198720425,"version":"3.50.1"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2012,6,4]],"date-time":"2012-06-04T00:00:00Z","timestamp":1338768000000},"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":[[2012,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Creating a national registry for bleeding disorders is a major step in establishing a National Hemophilia Care Program in all countries. Creating such a registry which would contain accurate and regularly updated data, including laboratory analysis confirmed by a reference laboratory established at the Syrian Hemophilia Society. Blood samples were drawn and analysed in the Society reference laboratory for the following screening tests: prothrombin time (<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content>), <jats:styled-content style=\"fixed-case\">APTT<\/jats:styled-content> and coagulation factor assays. Inhibitor detection and <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> RiCof were performed depending on the result of the screening tests. <jats:styled-content style=\"fixed-case\">HB<\/jats:styled-content>s Ag, anti\u2010<jats:styled-content style=\"fixed-case\">HCV<\/jats:styled-content>, anti\u2010<jats:styled-content style=\"fixed-case\">HIV<\/jats:styled-content> 1\u00a0+\u00a02 and syphilis tests were also performed to detect transfusion transmitted agents (<jats:styled-content style=\"fixed-case\">TTA<\/jats:styled-content>). Diagnosis of the bleeding disorder type was confirmed for 760 of these cases. Among the 760 confirmed patients, 82.5% had haemophilia. Among these, 89.6%were haemophilia A; 10.4% were haemophilia B; 8.3% had <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>; 9.2% had other rare bleeding disorders as follows: 1.2% <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency, 0.7% <jats:styled-content style=\"fixed-case\">FV<\/jats:styled-content> deficiency, 1.8% F1 deficiency, 0.4% <jats:styled-content style=\"fixed-case\">FX<\/jats:styled-content> deficiency, 1.4% platelets dysfunctions (mainly Glanzmann Thrombasthenia) and 3.7% had combined <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">FV<\/jats:styled-content> deficiency. Eighty (21.3%) cases of 375 screened for transfusion transmitted agents were positive for at least one infection: 0.5% were <jats:styled-content style=\"fixed-case\">HB<\/jats:styled-content>sAg positive, 19.7% were anti\u2010<jats:styled-content style=\"fixed-case\">HCV<\/jats:styled-content> positive, 0.8% had combined <jats:styled-content style=\"fixed-case\">HB<\/jats:styled-content>sAg and anti\u2010<jats:styled-content style=\"fixed-case\">HCV<\/jats:styled-content> positivity and 0.3% was anti\u2010Syphilis positive. All patients were negative for <jats:styled-content style=\"fixed-case\">HIV<\/jats:styled-content>1 and <jats:styled-content style=\"fixed-case\">HIV<\/jats:styled-content>2. The preliminary data presented here follow known data on haemophilia A, haemophilia B and <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> disease. This registry will certainly help in improving haemophilia care in Syria.<\/jats:p>","DOI":"10.1111\/j.1365-2516.2012.02862.x","type":"journal-article","created":{"date-parts":[[2012,6,5]],"date-time":"2012-06-05T14:45:08Z","timestamp":1338907508000},"page":"851-854","source":"Crossref","is-referenced-by-count":6,"title":["Registry of Hemophilia and other bleeding disorders in Syria"],"prefix":"10.1111","volume":"18","author":[{"given":"T.","family":"Ali","sequence":"first","affiliation":[{"name":"Immuno\u2010Hematology and Blood Transfusion Department, Faculty of Medicine Damascus University  Damascus Syria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. F.","family":"Schved","sequence":"additional","affiliation":[{"name":"Hemophilia Treatment Center, Hospital Saint\u2010Eloi Montpellier University Hospital  Montpellier France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,6,4]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01679.x"},{"key":"e_1_2_7_3_1","first-page":"1","article-title":"Registry of inherited bleeding disorders in Poland \u2013 current status and potential role of the HemoRec database","volume":"10","author":"Zdziarska J","year":"2010","journal-title":"Haemophilia"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02101.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01907.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02671.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00699.x"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02127.x"},{"key":"e_1_2_7_9_1","first-page":"136","article-title":"Inherited bleeding disorders: results from the Italian Regional Haemophilia Centre of Pescara","volume":"6","author":"Dragani A","year":"2008","journal-title":"Blood Transfus"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2003.00780.x"},{"key":"e_1_2_7_11_1","first-page":"1047","article-title":"The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977\u201399","volume":"2","author":"UK Haemophilia Centre Doctors\u2018Organisation","journal-title":"J Thromb Haemost"},{"issue":"2","key":"e_1_2_7_12_1","first-page":"s45","article-title":"National and international registries of rare bleeding disorders","volume":"6","author":"Peyvandi F","year":"2008","journal-title":"Blood Transfus"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2003.t01-1-00553.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00616.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1590\/S1020-49892003000200013"},{"key":"e_1_2_7_16_1","first-page":"107","article-title":"Pr\u00e9valence des infections transmissibles par transfusion chez les donneurs de sang au centre de transfusion sanguine de l\u2018universit\u00e9 de Damas et l\u2018importance des mesures de la s\u00e9curit\u00e9 transfusionnelle","volume":"26","author":"Ali T","year":"2010","journal-title":"Damascus Univ J Health Sci"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2516.2012.02862.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-2516.2012.02862.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T09:34:29Z","timestamp":1698658469000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-2516.2012.02862.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,6,4]]},"references-count":15,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2012,11]]}},"alternative-id":["10.1111\/j.1365-2516.2012.02862.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-2516.2012.02862.x","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,6,4]]}}}