{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T11:40:01Z","timestamp":1783942801884,"version":"3.55.0"},"reference-count":56,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":4362,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1994,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Congenital afibrinogenemia is a rare disorder with unusual clinical manifestations. The disease is inherited as an autosomal recessive trait and consanguinity is common among affected families. Clinical manifestations range from minimal bleeding to catastrophic hemorrhage. Congenitally afibrinogenemic patients seem to be peculiarly susceptible to spontaneous rupture of the spleen. Coagulation tests which depend on clot formation as an end point may be infinitely prolonged and abnormalities of platelet function are usually present. The diagnosis is established by demonstrating trace or no immunoreactive fibrinogen. The disease is caused by markedly reduced or absent synthesis of fibrinogen by liver cells, but the genetic defect remains unknown. Bleeding episodes can be effectively treated with cryoprecipitate. Purified virally inactivated fibrinogen concentrates have been used in Europe and may soon be widely available. \u00a9 1994 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830460416","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:42:43Z","timestamp":1172065363000},"page":"343-347","source":"Crossref","is-referenced-by-count":84,"title":["Congenital afibrinogenemia"],"prefix":"10.1002","volume":"46","author":[{"given":"Hamid","family":"Al\u2010Mondhiry","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. Christopher","family":"Ehmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00291325"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.82.8.2344"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4615-3806-6_3"},{"key":"e_1_2_1_5_2","first-page":"182","volume-title":"Hemostasis and Thrombosis","author":"Hawiger J","year":"1987"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198408233110804"},{"key":"e_1_2_1_7_2","first-page":"565","article-title":"A major coronary risk factor","volume":"35","author":"Stone MC","year":"1985","journal-title":"J R Coil Gen Pract"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1987.03400090067035"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(90)92339-4"},{"key":"e_1_2_1_10_2","first-page":"240","article-title":"Uber Faserstoffmangel im Blut bci einem Falle von Haemophilie","volume":"132","author":"Rabe F","year":"1920","journal-title":"Dtsch Arch Kli Med"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/S0308-2261(21)00068-0","article-title":"Congenital abnormalities of fibrinogen","volume":"8","author":"Beck EA","year":"1979","journal-title":"Clin Haemat"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-1004999"},{"key":"e_1_2_1_13_2","first-page":"141","volume-title":"Disorders of Hemostasis","author":"Forbes CD","year":"1991"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1980.tb00137.x"},{"key":"e_1_2_1_15_2","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1093\/oxfordjournals.bmb.a071444","article-title":"Disorders of plasma fibrinogen synthesis","volume":"33","author":"Flute PT","year":"1977","journal-title":"Br Med Bull"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1159\/000205692"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(84)91264-6"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1159\/000209109"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0028-2243(85)90046-2"},{"key":"e_1_2_1_20_2","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1055\/s-0038-1649265","article-title":"Inherited bleeding syndromes in Iraq","volume":"37","author":"Aj\u2010Mondhiry H","year":"1977","journal-title":"Thromb Haemostas"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9378(85)90354-0"},{"key":"e_1_2_1_22_2","first-page":"1422","volume-title":"Wintrobe's Clinical Hematology","author":"Bithell TC","year":"1993"},{"key":"e_1_2_1_23_2","first-page":"1474","volume-title":"Hematology","author":"Gralnick HR","year":"1990"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(94)90123-6"},{"key":"e_1_2_1_25_2","first-page":"555","article-title":"Afibrinogenemia with cerebral hematoma","volume":"131","author":"Montgomery R","year":"1977","journal-title":"Am J Dis Child"},{"key":"e_1_2_1_26_2","first-page":"1438","article-title":"A case of congenital afibrinogenemia with abortion, intracranial hemorrhage and peritonitis","volume":"12","author":"Matsuno K","year":"1977","journal-title":"Jpn J Clin Hematol"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-0528.1991.tb13464.x"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb04491.x"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830410111"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01633614"},{"key":"e_1_2_1_31_2","first-page":"170","article-title":"Influence of fibrinogen on the aggregation of washed human platelets induced by adenosine diphosphate, thrombin, collagen and adrenalin","volume":"84","author":"Solum N","year":"1965","journal-title":"Scand J Clin Lab Invest (suppl)"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1620\/tjem.96.15"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197108122850703"},{"key":"e_1_2_1_34_2","doi-asserted-by":"publisher","DOI":"10.1038\/202605a0"},{"key":"e_1_2_1_35_2","first-page":"361","article-title":"Platelet function in congenital afibrinogenemia","volume":"14","author":"G\u00fcgler E","year":"1965","journal-title":"Throm Diath Haemorrh"},{"key":"e_1_2_1_36_2","first-page":"37","article-title":"Plasma protein requirement for human platelet aggregation","volume":"42","author":"Bang NU","year":"1970","journal-title":"Thromb Diath Haemorrh (suppl)"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1968.tb01528.x"},{"key":"e_1_2_1_38_2","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1182\/blood.V70.1.221.221","article-title":"Fibrinogen\u2010independent aggregation and deaggregation of human platelets: Studies in two afibrinogenemic patients","volume":"70","author":"Cattaneo M","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_39_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109425"},{"key":"e_1_2_1_40_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(78)91308-9"},{"key":"e_1_2_1_41_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(87)91098-1"},{"key":"e_1_2_1_42_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197106)27:6<1313::AID-CNCR2820270606>3.0.CO;2-W"},{"key":"e_1_2_1_43_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1972.tb05716.x"},{"key":"e_1_2_1_44_2","volume-title":"The Molecular Basis of Blood Disease","author":"Crabtree GR","year":"1987"},{"key":"e_1_2_1_45_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)91001-X"},{"key":"e_1_2_1_46_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1979.03290420016016"},{"key":"e_1_2_1_47_2","first-page":"158","article-title":"Management of the hereditary coagulation disorders","volume":"8","author":"Mason DY","year":"1971","journal-title":"Semin Hematol"},{"key":"e_1_2_1_48_2","first-page":"59","volume-title":"Hemorrhage and thrombosis: A Practical Clinical Guide","author":"Salzman EW","year":"1965"},{"key":"e_1_2_1_49_2","volume-title":"Hemostasis and Thrombosis","author":"Ratnoff OD","year":"1987"},{"key":"e_1_2_1_50_2","volume-title":"Hematology","author":"Menitove JE","year":"1990"},{"issue":"1","key":"e_1_2_1_51_2","first-page":"156a","article-title":"Use of a new solvent\u2010detergent\/heat\u2010treated fibrinogen concentrate in a child with afibrinogenemia","volume":"82","author":"Broxson EH","year":"1993","journal-title":"Blood"},{"key":"e_1_2_1_52_2","doi-asserted-by":"publisher","DOI":"10.1177\/000992288802701106"},{"key":"e_1_2_1_53_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112430"},{"key":"e_1_2_1_54_2","first-page":"365","article-title":"Effect of DDAVP on primary hemostasis with congenital afibrinogenemia","volume":"58","author":"Taki M","year":"1987","journal-title":"Thromb Haemost"},{"key":"e_1_2_1_55_2","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(92)90088-R"},{"key":"e_1_2_1_56_2","doi-asserted-by":"publisher","DOI":"10.1159\/000204533"},{"key":"e_1_2_1_57_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(61)90057-2"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830460416","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830460416","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T21:39:50Z","timestamp":1696023590000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830460416"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1994,8]]},"references-count":56,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1994,8]]}},"alternative-id":["10.1002\/ajh.2830460416"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830460416","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1994,8]]}}}