{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T23:40:22Z","timestamp":1793749222926,"version":"4.6.1"},"reference-count":54,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,6,23]],"date-time":"2006-06-23T00:00:00Z","timestamp":1151020800000},"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":[[2006,7]]},"abstract":"<jats:p><jats:bold>Summary.\u2002<\/jats:bold> A thorough review of the literature and of personal files has allowed the gathering of 81 patients with rare congenital bleeding disorders and thrombotic phenomena. Sixteen of these patients had congenital afibrinogenemia, eight involved factor V deficiency, 20 factor VII defects, 33 factor XI deficiencies and only one, a factor XIII defect. Altogether 42 patients showed arterial thrombosis (myocardial infarction [MI] in 28 cases; ischemic stroke in 4; arterial occlusion in 8; 2 patients with disseminated intravascular coagulation (DIC)). Ages varied between 13 and 74. Twenty\u2010two patients were males and 16 females. In four cases, sex was not reported. There were three fatalities: two after a MI and one because of heart failure. With regard to venous thrombosis: 9 patients had pulmonary embolism, 15 patients had deep vein thrombosis, 9 patients had both pulmonary embolism and deep vein thrombosis; 1 patient had superficial vein thrombosis, whereas, 5 cases had an unusual site venous thrombosis (two portal systems, two cerebral sinuses, one inferior vena cava) for a total of 39 cases. Age varied between 3 and 86. In this case, 20 patients were males and 17 were females. In two cases, sex was not reported. There were three fatalities: two because of pulmonary embolism and one because of inferior vena cava thrombosis. The fact that thrombosis has never been described in patients with factor II or factor X seems to underscore the central antithrombotic role that these two factors have in the coagulation system.<\/jats:p>","DOI":"10.1111\/j.1365-2516.2006.01299.x","type":"journal-article","created":{"date-parts":[[2006,6,23]],"date-time":"2006-06-23T14:55:42Z","timestamp":1151074542000},"page":"345-351","source":"Crossref","is-referenced-by-count":79,"title":["Arterial and venous thrombosis in rare congenital bleeding disorders: a critical review"],"prefix":"10.1111","volume":"12","author":[{"given":"A.","family":"GIROLAMI","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"RUZZON","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"TEZZA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"SCANDELLARI","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"VETTORE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"GIROLAMI","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,6,23]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00906.x"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-005-2227-3"},{"key":"e_1_2_5_4_2","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1160\/TH04-03-0148","article-title":"Thrombotic complications in patients with hereditary bleeding disorders","volume":"92","author":"Franchini M","year":"2004","journal-title":"Thromb Haemost"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.03186.x"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-006-5732-0"},{"key":"e_1_2_5_7_2","first-page":"771","article-title":"Gastrectomy for a patient with congenital factor VII deficiency\u2014a case report]","volume":"91","author":"Nakagawa K","year":"1990","journal-title":"Nippon Geka Gakkai Zasshi"},{"key":"e_1_2_5_8_2","first-page":"387","article-title":"Factor VII Padua: a congenital coagulation disorder due to an abnormal factor VII with a peculiar activation pattern","volume":"91","author":"Girolami A","year":"1978","journal-title":"J Lab Clin Med"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(64)90003-8"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1538-7836.2003.00395.x"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1998.00181.x"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198307000-00004"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1097\/00001721-199509000-00008"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1159\/000205692"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1177\/000331979304400913"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(61)90057-2"},{"key":"e_1_2_5_17_2","first-page":"1820","article-title":"Portal vein thrombosis revealing congenital afibrinogenemia","volume":"21","author":"Drai E","year":"1992","journal-title":"Presse Med"},{"key":"e_1_2_5_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(01)00247-X"},{"key":"e_1_2_5_19_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.27.12.2325"},{"key":"e_1_2_5_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830410111"},{"key":"e_1_2_5_21_2","doi-asserted-by":"publisher","DOI":"10.1159\/000209109"},{"key":"e_1_2_5_22_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01681.x"},{"key":"e_1_2_5_23_2","first-page":"597","article-title":"Congenital afibrinogenemia. 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