{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:32:36Z","timestamp":1790814756686,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,9,19]],"date-time":"2016-09-19T00:00:00Z","timestamp":1474243200000},"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":[[2016,11]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>No evidence\u2010based guidelines for the management of patients suffering from afibrinogenaemia and hypofibrinogenaemia are available.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim and method<\/jats:title><jats:p>The aim of this study was to harmonize patient's care among invited haemophilia experts from Belgium, France and Switzerland. A Delphi\u2010like methodology was used to reach a consensus on: prophylaxis, bleeding, surgery, pregnancy and thrombosis management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The main final statements are as follows: (i) a secondary fibrinogen prophylaxis should be started after a first life\u2010threatening bleeding in patients with afibrinogenaemia; (ii) during prophylaxis the target trough fibrinogen level should be 0.5 g L<jats:sup>\u22121<\/jats:sup>; (iii) if an adaptation of dosage is required, the frequency of infusions rather than the fibrinogen amount should be modified; (iv) afibrinogenaemic patients undergoing a surgery at high bleeding risk should receive fibrinogen concentrates regardless of the personal or family history of bleeding; (v) moderate hypofibrinogenaemic patients (i.e. \u22650.5 g L<jats:sup>\u22121<\/jats:sup>) without previous bleeding (despite haemostatic challenges) undergoing a surgery at low bleeding risk may not receive fibrinogen concentrates as prophylaxis; (vi) monitoring the trough fibrinogen levels should be performed at least once a month throughout the pregnancy and a foetal growth and placenta development close monitoring by ultrasound is recommended; (vii) fibrinogen replacement should be started concomitantly to the introduction of anticoagulation in afibrinogenaemic patients suffering from a venous thromboembolic event; and (viii) low\u2010molecular\u2010weight heparin is the anticoagulant of choice in case of venous thromboembolism.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The results of this initiative should help clinicians in the difficult management of patients with congenital fibrinogen disorders.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13061","type":"journal-article","created":{"date-parts":[[2016,9,19]],"date-time":"2016-09-19T11:32:41Z","timestamp":1474284761000},"page":"898-905","source":"Crossref","is-referenced-by-count":45,"title":["Management of congenital quantitative fibrinogen disorders: a Delphi consensus"],"prefix":"10.1111","volume":"22","author":[{"given":"A.","family":"Casini","sequence":"first","affiliation":[{"name":"Division of Angiology and Haemostasis University Hospitals and Faculty of Medicine Geneva Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"de Moerloose","sequence":"additional","affiliation":[{"name":"Division of Angiology and Haemostasis University Hospitals and Faculty of Medicine Geneva Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Congenital Fibrinogen Disorders Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,9,19]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0036-1571340"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-08-532820"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70004-5"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04653.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02014.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0036-1571339"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00944.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13058"},{"key":"e_1_2_8_10_1","first-page":"575","article-title":"Principles of treatment and update of recommendations for the management of haemophilia and congenital bleeding disorders in Italy","volume":"12","author":"Rocino A","year":"2014","journal-title":"Blood Transfus"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197306142882405"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.311.7001.376"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12189"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1258\/135581906778476553"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2648.2003.02537.x"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH06-01-0020"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2010.12.001"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2015.11.026"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01681.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01340.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04424.x"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03076.x"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03633.x"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2010.02612.x"},{"issue":"2","key":"e_1_2_8_25_1","first-page":"PO502:445","article-title":"Fibrinogen deficiency: preliminary results of the PRORBDD project. Abstracts of the XXV Congress of the International Society on Thrombosis and Haemostasis","volume":"13","author":"Peyvandi F","year":"2015","journal-title":"J Thromb Haemost"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1349222"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02118.x"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajem.2010.05.016"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(01)00247-X"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2004.08.003"},{"issue":"2","key":"e_1_2_8_31_1","first-page":"PO484:436","article-title":"Pharmacokinetic and pharmacodynamic results of a multicenter, open\u2010label, single arm study with a fibrinogen concentrate (FGTW) in subjects with afibrinogenemia. Abstracts of the XXV Congress of the International Society on Thrombosis and Haemostasis","volume":"13","author":"Khayat C","year":"2015","journal-title":"J Thromb Haemost"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.2176\/nmc.51.780"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0265021508003785"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12584"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.2174\/1389450054546006"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64923-1"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02363.x"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.01993.x"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.siny.2011.07.006"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20802"},{"key":"e_1_2_8_41_1","first-page":"52","article-title":"Constitutional hypofibrinogenemia associated with third trimester hemorrhage","volume":"16","author":"McRoyan DK","year":"1986","journal-title":"Ann Clin Lab Sci"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2011.03.012"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-015-1325-0"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00252836"},{"issue":"2","key":"e_1_2_8_45_1","first-page":"OR432:261","article-title":"Hereditary afibrinogenemia: long term observation of a highly thrombogenic condition and its management. Abstracts of the XXV Congress of the International Society on Thrombosis and Haemostasis","volume":"13","author":"Nagler M","year":"2015","journal-title":"J Thromb Haemost"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12234"},{"key":"e_1_2_8_47_1","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1097\/MBC.0000000000000106","article-title":"Coexistence of hypofibrinogenemia and factor V Leiden mutation: is the balance shifted to thrombosis?","volume":"25","author":"Miljic P","year":"2014","journal-title":"Blood Coagul Fibrinolysis"},{"key":"e_1_2_8_48_1","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1055\/s-0037-1614201","article-title":"Lepirudin therapy for thrombotic complications in congenital afibrinogenaemia","volume":"91","author":"Schuepbach RA","year":"2004","journal-title":"Thromb Haemost"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12734"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1177\/0218492312455180"},{"key":"e_1_2_8_51_1","first-page":"e104","article-title":"Afibrinogenaemia: a rare cause of young myocardial infarct","volume":"49","author":"Kumar N","year":"2008","journal-title":"Singapore Med J"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13061","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13061","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.13061","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T20:26:48Z","timestamp":1695500808000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.13061"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9,19]]},"references-count":50,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2016,11]]}},"alternative-id":["10.1111\/hae.13061"],"URL":"https:\/\/doi.org\/10.1111\/hae.13061","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,19]]}}}