{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T23:40:33Z","timestamp":1793749233476,"version":"4.6.1"},"reference-count":97,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,7,15]],"date-time":"2025-07-15T00:00:00Z","timestamp":1752537600000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003407","name":"Ministero dell\u2019Istruzione, dell\u2019Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100021856","name":"Ministero dell'Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100021856","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.jp","rpthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Research and Practice in Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2025,7]]},"DOI":"10.1016\/j.rpth.2025.102979","type":"journal-article","created":{"date-parts":[[2025,7,22]],"date-time":"2025-07-22T23:14:36Z","timestamp":1753226076000},"page":"102979","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":10,"title":["The thrombotic paradox in congenital fibrinogen deficiencies: from pathophysiology to practice"],"prefix":"10.1016","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5004-4456","authenticated-orcid":false,"given":"Samin","family":"Mohsenian","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Casini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flora","family":"Peyvandi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.rpth.2025.102979_bib1","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1055\/s-0033-1349222","article-title":"Congenital fibrinogen disorders: an update","volume":"39","author":"de Moerloose","year":"2013","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.rpth.2025.102979_bib2","doi-asserted-by":"crossref","first-page":"2711","DOI":"10.3390\/ijms18122711","article-title":"Fibrinogen as a pleiotropic protein causing human diseases: the mutational burden of A\u03b1, B\u03b2, and \u03b3 chains","volume":"18","author":"Paraboschi","year":"2017","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib3","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1182\/blood.V6.1.84.84","article-title":"Conversion factors and accelerators in the formation of thrombin","volume":"6","author":"Stefanini","year":"1951","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib4","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1055\/s-0036-1571340","article-title":"Laboratory and genetic investigation of mutations accounting for congenital fibrinogen disorders","volume":"42","author":"Neerman-Arbez","year":"2016","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.rpth.2025.102979_bib5","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1016\/S0002-9440(10)64923-1","article-title":"Fibrinogen stabilizes placental-maternal attachment during embryonic development in the mouse","volume":"160","author":"Iwaki","year":"2002","journal-title":"Am J Pathol"},{"key":"10.1016\/j.rpth.2025.102979_bib6","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.thromres.2013.08.023","article-title":"The role of fibrin matrices and tissue factor in early-term trophoblast proliferation and spreading","volume":"132","author":"Snir","year":"2013","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib7","doi-asserted-by":"crossref","first-page":"3127","DOI":"10.1182\/blood.2020009472","article-title":"Clinical phenotype, fibrinogen supplementation, and health-related quality of life in patients with afibrinogenemia","volume":"137","author":"Casini","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib8","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1007\/s11239-015-1325-0","article-title":"Thromboembolic events in patients with severe inherited fibrinogen deficiency","volume":"42","author":"Rottenstreich","year":"2016","journal-title":"J Thromb Thrombolysis"},{"key":"10.1016\/j.rpth.2025.102979_bib9","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.3390\/jcm11041083","article-title":"Heterogeneity of genotype-phenotype in congenital hypofibrinogenemia-a review of case reports associated with bleeding and thrombosis","volume":"11","author":"Brunclikova","year":"2022","journal-title":"J Clin Med"},{"key":"10.1016\/j.rpth.2025.102979_bib10","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1055\/a-2094-7191","article-title":"Fibrinogen Bonn (p. Arg510Cys) in the A\u03b1-chain is associated with high risk of venous thrombosis","volume":"43","author":"Iva\u0161kevi\u010dius","year":"2023","journal-title":"Hamostaseologie"},{"key":"10.1016\/j.rpth.2025.102979_bib11","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1160\/TH08-11-0777","article-title":"Hypodysfibrinogenemia causing mild bleeding and thrombotic complications in a compound heterozygote of A\u03b1IVS4+1G>T mutation and A\u03b14841delC truncation (A\u03b1Perth)","volume":"101","author":"Jayo","year":"2009","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib12","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1182\/bloodadvances.2023012186","article-title":"Congenital fibrinogen disorders: a retrospective clinical and genetic analysis of the Prospective Rare Bleeding Disorders Database","volume":"8","author":"Mohsenian","year":"2024","journal-title":"Blood Adv"},{"key":"10.1016\/j.rpth.2025.102979_bib13","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1111\/jth.14216","article-title":"Diagnosis and classification of congenital fibrinogen disorders: communication from the SSC of the ISTH","volume":"16","author":"Casini","year":"2018","journal-title":"J Thromb Haemost"},{"issue":"suppl 2","key":"10.1016\/j.rpth.2025.102979_bib14","doi-asserted-by":"crossref","first-page":"S7","DOI":"10.1016\/S0049-3848(13)70004-5","article-title":"Epidemiology and treatment of congenital fibrinogen deficiency","volume":"130","author":"Peyvandi","year":"2012","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib15","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1159\/000452864","article-title":"Thrombosis in inherited fibrinogen disorders","volume":"44","author":"Korte","year":"2017","journal-title":"Transfus Med Hemother"},{"key":"10.1016\/j.rpth.2025.102979_bib16","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1055\/s-0036-1571339","article-title":"Clinical features and management of congenital fibrinogen deficiencies","volume":"42","author":"Casini","year":"2016","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.rpth.2025.102979_bib17","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1160\/TH16-02-0082","article-title":"Thromboembolism in patients with congenital afibrinogenaemia. Long-term observational data and systematic review","volume":"116","author":"Nagler","year":"2016","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib18","doi-asserted-by":"crossref","first-page":"1687","DOI":"10.1111\/j.1538-7836.2011.04424.x","article-title":"Fibrinogen replacement therapy for congenital fibrinogen deficiency","volume":"9","author":"Bornikova","year":"2011","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib19","first-page":"235","article-title":"Erythrocyte hyperaggregation and thrombogenic dysfibrinogenemia","volume":"18","author":"Nguyen","year":"1998","journal-title":"Clin Hemorheol Microcirc"},{"key":"10.1016\/j.rpth.2025.102979_bib20","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1046\/j.1365-2141.1999.01681.x","article-title":"Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia","volume":"107","author":"Lak","year":"1999","journal-title":"Br J Haematol"},{"key":"10.1016\/j.rpth.2025.102979_bib21","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.transci.2004.08.003","article-title":"Efficacy and tolerability of a pasteurised human fibrinogen concentrate in patients with congenital fibrinogen deficiency","volume":"32","author":"Kreuz","year":"2005","journal-title":"Transfus Apher Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib22","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1097\/01.mbc.0000224841.48463.be","article-title":"Inherited abnormalities of fibrinogen: 10-year clinical experience of an Italian group","volume":"17","author":"Santacroce","year":"2006","journal-title":"Blood Coagul Fibrinolysis"},{"key":"10.1016\/j.rpth.2025.102979_bib23","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1097\/MBC.0b013e328331e6db","article-title":"Inherited dysfibrinogenemia: clinical phenotypes associated with five different fibrinogen structure defects","volume":"21","author":"Miesbach","year":"2010","journal-title":"Blood Coagul Fibrinolysis"},{"key":"10.1016\/j.rpth.2025.102979_bib24","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1111\/bjh.12085","article-title":"Clinical phenotype, laboratory features and genotype of 35 patients with heritable dysfibrinogenaemia","volume":"160","author":"Shapiro","year":"2013","journal-title":"Br J Haematol"},{"key":"10.1016\/j.rpth.2025.102979_bib25","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1182\/blood-2014-06-582866","article-title":"Natural history of patients with congenital dysfibrinogenemia","volume":"125","author":"Casini","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib26","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1016\/j.bcmd.2015.06.002","article-title":"Clinical features and molecular basis of 102 Chinese patients with congenital dysfibrinogenemia","volume":"55","author":"Zhou","year":"2015","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.rpth.2025.102979_bib27","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/j.thromres.2015.05.007","article-title":"Molecular characterization of 7 patients affected by dys- or hypo-dysfibrinogenemia: identification of a novel mutation in the fibrinogen Bbeta chain causing a gain of glycosylation","volume":"136","author":"Asselta","year":"2015","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib28","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1160\/TH14-07-0629","article-title":"Clinical and molecular characterisation of 21 patients affected by quantitative fibrinogen deficiency","volume":"113","author":"Asselta","year":"2015","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib29","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1309\/AJCPDQL7LXMY7SHW","article-title":"Paradoxical bleeding and thrombosis in a patient with afibrinogenemia and fibrinogen Mumbai mutation","volume":"143","author":"Mukaddam","year":"2015","journal-title":"Am J Clin Pathol"},{"key":"10.1016\/j.rpth.2025.102979_bib30","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.thromres.2015.11.008","article-title":"Fibrin clot structure in patients with congenital dysfibrinogenaemia","volume":"137","author":"Casini","year":"2016","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib31","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1111\/vox.12424","article-title":"Post-authorization safety study of Clottafact\u00ae, a triply secured fibrinogen concentrate in congenital afibrinogenemia. A prospective observational study","volume":"111","author":"N\u00e9grier","year":"2016","journal-title":"Vox Sang"},{"key":"10.1016\/j.rpth.2025.102979_bib32","first-page":"247","article-title":"Identification of novel mutations in patients with fibrinogen disorders and genotype\/phenotype correlations","volume":"17","author":"Chinni","year":"2019","journal-title":"Blood Transfus"},{"key":"10.1016\/j.rpth.2025.102979_bib33","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1002\/rth2.12127","article-title":"Identification and characterization of novel mutations implicated in congenital fibrinogen disorders","volume":"2","author":"Smith","year":"2018","journal-title":"Res Pract Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib34","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1111\/ejh.13296","article-title":"Risk of bleeding and thrombosis in inherited qualitative fibrinogen disorders","volume":"103","author":"Castaman","year":"2019","journal-title":"Eur J Haematol"},{"key":"10.1016\/j.rpth.2025.102979_bib35","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1111\/hae.14230","article-title":"Efficacy and safety of fibrinogen concentrate for on-demand treatment of bleeding and surgical prophylaxis in paediatric patients with congenital fibrinogen deficiency","volume":"27","author":"Djambas Khayat","year":"2021","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib36","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.thromres.2019.08.012","article-title":"Genetic and clinical characterization of congenital fibrinogen disorders in Polish patients: identification of three novel fibrinogen gamma chain mutations","volume":"182","author":"Wypasek","year":"2019","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib37","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1002\/rth2.12433","article-title":"Fibrinogen concentrate for bleeding in patients with congenital fibrinogen deficiency: observational study of efficacy and safety for prophylaxis and treatment","volume":"4","author":"Lasky","year":"2020","journal-title":"Res Pract Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib38","doi-asserted-by":"crossref","DOI":"10.1016\/j.bcmd.2020.102489","article-title":"Identification and characterization of novel mutations in Chinese patients with congenital fibrinogen disorders","volume":"86","author":"Zhou","year":"2021","journal-title":"Blood Cells Mol Dis"},{"key":"10.1016\/j.rpth.2025.102979_bib39","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1111\/hae.14579","article-title":"Clinical, biological, and genetic features in an afibrinogenemia patient series in Algeria","volume":"28","author":"Hadjali-Saichi","year":"2022","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib40","doi-asserted-by":"crossref","first-page":"2126","DOI":"10.1016\/j.jtha.2023.04.035","article-title":"Obstetrical complications in hereditary fibrinogen disorders: the Fibrinogest study","volume":"21","author":"Hugon-Rodin","year":"2023","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib41","article-title":"Clinical and genetic characterization of 51 patients with congenital fibrinogen disorders from China","author":"Cai","year":"2025","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib42","doi-asserted-by":"crossref","DOI":"10.1016\/j.thromres.2025.109317","article-title":"Phenotype, genotype, and laboratory assessment of congenital fibrinogen disorders: data from the Rare Bleeding disorders in the Netherlands study","volume":"249","author":"Haisma","year":"2025","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib43","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.thromres.2021.06.020","article-title":"Differences in the biochemical composition of three plasma derived human fibrinogen concentrates","volume":"205","author":"Neisser-Svae","year":"2021","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib44","doi-asserted-by":"crossref","first-page":"2021","DOI":"10.1182\/blood.2020010116","article-title":"How I treat dysfibrinogenemia","volume":"138","author":"Casini","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib45","series-title":"Index of variant human fibrinogens","author":"Ebert","year":"1994"},{"key":"10.1016\/j.rpth.2025.102979_bib46","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1055\/s-0038-1653741","article-title":"Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen","volume":"73","author":"Haverkate","year":"1995","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib47","doi-asserted-by":"crossref","first-page":"585","DOI":"10.3109\/00365513.2013.826818","article-title":"Dusart Syndrome in a Scandinavian family characterized by arterial and venous thrombosis at young age","volume":"73","author":"Ramanathan","year":"2013","journal-title":"Scand J Clin Lab Invest"},{"key":"10.1016\/j.rpth.2025.102979_bib48","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1055\/s-0036-1581103","article-title":"Severe thrombotic complications in congenital afibrinogenemia: a pathophysiological and management dilemma","volume":"42","author":"Santoro","year":"2016","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.rpth.2025.102979_bib49","doi-asserted-by":"crossref","first-page":"192","DOI":"10.3390\/ijms19010192","article-title":"Clinical consequences and molecular bases of low fibrinogen levels","volume":"19","author":"Neerman-Arbez","year":"2018","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib50","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1055\/s-0037-1613536","article-title":"Antithrombin I. Inhibition of thrombin generation in plasma by fibrin formation","volume":"89","author":"Mosesson","year":"2003","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib51","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1055\/s-0037-1613195","article-title":"Inhibition of thrombin generation in plasma by fibrin formation (antithrombin I)","volume":"88","author":"de Bosch","year":"2002","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib52","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1055\/s-0038-1648947","article-title":"The influence of fibrinogen and fibrin on thrombin generation--evidence for feedback activation of the clotting system by clot bound thrombin","volume":"72","author":"Kumar","year":"1994","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib53","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0049-3848(01)00247-X","article-title":"Embolized ischemic lesions of toes in an afibrinogenemic patient: possible relevance to in vivo circulating thrombin","volume":"102","author":"Dupuy","year":"2001","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib54","doi-asserted-by":"crossref","first-page":"499","DOI":"10.5858\/2002-126-0499-LDOD","article-title":"Laboratory diagnosis of dysfibrinogenemia","volume":"126","author":"Cunningham","year":"2002","journal-title":"Arch Pathol Lab Med"},{"key":"10.1016\/j.rpth.2025.102979_bib55","doi-asserted-by":"crossref","first-page":"1272","DOI":"10.1172\/JCI112430","article-title":"von Willebrand factor interaction with the glycoprotein IIb\/IIa complex. Its role in platelet function as demonstrated in patients with congenital afibrinogenemia","volume":"77","author":"De Marco","year":"1986","journal-title":"J Clin Invest"},{"key":"10.1016\/j.rpth.2025.102979_bib56","doi-asserted-by":"crossref","first-page":"655","DOI":"10.3390\/ijms22020655","article-title":"Venous thrombosis and thrombocyte activity in zebrafish models of quantitative and qualitative fibrinogen disorders","volume":"22","author":"Fish","year":"2021","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib57","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1016\/S0006-3495(96)79596-6","article-title":"Fibrinogen Dusart: electron microscopy of molecules, fibers and clots, and viscoelastic properties of clots","volume":"70","author":"Collet","year":"1996","journal-title":"Biophys J"},{"key":"10.1016\/j.rpth.2025.102979_bib58","doi-asserted-by":"crossref","first-page":"1422","DOI":"10.1111\/j.1538-7836.2008.03013.x","article-title":"A new electrophoretic variant of fibrinogen associated with venous thromboembolism, fibrinogen Bordeaux A\u03b1 Arg439\u2192Cys","volume":"6","author":"Hanss","year":"2008","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib59","doi-asserted-by":"crossref","first-page":"1597","DOI":"10.3390\/ijms19061597","article-title":"Human fibrinogen: molecular and genetic aspects of congenital disorders","volume":"19","author":"Tiscia","year":"2018","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib60","doi-asserted-by":"crossref","first-page":"1874","DOI":"10.1172\/JCI106680","article-title":"Functional evaluation of an inherited abnormal fibrinogen: fibrinogen \u201cBaltimore\u201d","volume":"50","author":"Beck","year":"1971","journal-title":"J Clin Invest"},{"key":"10.1016\/j.rpth.2025.102979_bib61","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1111\/jth.12229","article-title":"Fibrin(ogen) and thrombotic disease","volume":"11","author":"Ari\u00ebns","year":"2013","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib62","doi-asserted-by":"crossref","first-page":"3709","DOI":"10.1182\/blood.V84.11.3709.bloodjournal84113709","article-title":"A correlation between thrombotic disease and a specific fibrinogen abnormality (A alpha 554 Arg-->Cys) in two unrelated kindred, Dusart and Chapel Hill III","volume":"84","author":"Wada","year":"1994","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib63","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1055\/s-0037-1614006","article-title":"Fibrinogen Caracas V, an abnormal fibrinogen with an A\u03b1 532 Ser\u2192Cys substitution associated with thrombosis","volume":"84","author":"Marchi","year":"2000","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib64","first-page":"575","article-title":"[Association of dysfibrinogenemia and thrombosis. Apropos of a family (Fibrinogen Melun) and review of the literature]","volume":"146","author":"Bentolila","year":"1995","journal-title":"Ann Med Interne (Paris)"},{"key":"10.1016\/j.rpth.2025.102979_bib65","doi-asserted-by":"crossref","first-page":"4390","DOI":"10.1016\/S0021-9258(18)89277-8","article-title":"Characterization of fibrinogen New York 1. A dysfunctional fibrinogen with a deletion of B beta(9-72) corresponding exactly to exon 2 of the gene","volume":"260","author":"Liu","year":"1985","journal-title":"J Biol Chem"},{"key":"10.1016\/j.rpth.2025.102979_bib66","doi-asserted-by":"crossref","first-page":"3478","DOI":"10.1073\/pnas.89.8.3478","article-title":"Abnormal fibrinogens IJmuiden (B beta Arg14----Cys) and Nijmegen (B beta Arg44----Cys) form disulfide-linked fibrinogen-albumin complexes","volume":"89","author":"Koopman","year":"1992","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.rpth.2025.102979_bib67","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1055\/s-0038-1647646","article-title":"Fibrinogen Nijmegen: congenital dysfibrinogenemia associated with impaired t-PA mediated plasminogen activation and decreased binding of t-PA","volume":"60","author":"Engesser","year":"1988","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib68","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1172\/JCI115841","article-title":"Molecular basis of fibrinogen Naples associated with defective thrombin binding and thrombophilia. Homozygous substitution of B beta 68 Ala----Thr","volume":"90","author":"Koopman","year":"1992","journal-title":"J Clin Invest"},{"key":"10.1016\/j.rpth.2025.102979_bib69","doi-asserted-by":"crossref","first-page":"e162","DOI":"10.1016\/j.thromres.2010.04.011","article-title":"Coexisting dysfibrinogenemia (\u03b3Arg275His) and FV Leiden associated with thrombosis (Fibrinogen Crete)","volume":"126","author":"Travlou","year":"2010","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib70","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":"Milji\u0107","year":"2014","journal-title":"Blood Coagul Fibrinolysis"},{"key":"10.1016\/j.rpth.2025.102979_bib71","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1097\/MBC.0000000000000790","article-title":"Recurrent superficial venous thrombophlebitis because of mutations in the protein C and fibrinogen genes in a young Argentinian female","volume":"30","author":"Guglielmone","year":"2019","journal-title":"Blood Coagul Fibrinolysis"},{"key":"10.1016\/j.rpth.2025.102979_bib72","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1186\/s12959-018-0162-8","article-title":"Combined life-threatening thromboses and hemorrhages in a patient with afibrinogenemia and antithrombin deficiency","volume":"16","author":"Le Quellec","year":"2018","journal-title":"Thromb J"},{"key":"10.1016\/j.rpth.2025.102979_bib73","doi-asserted-by":"crossref","first-page":"e380","DOI":"10.1111\/hae.12234","article-title":"Pulmonary embolism in a patient with congenital afibrinogenemia","volume":"19","author":"Chapin","year":"2013","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib74","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1111\/j.1365-2516.2007.01564.x","article-title":"Central venous catheter-related thrombosis after replacement therapy for intracranial bleeding in a patient with afibrinogenaemia","volume":"14","author":"Matsumoto","year":"2008","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib75","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1053\/j.jvca.2004.11.021","article-title":"Case 1-2005: cardiac surgery in congenital afibrinogenemia with thrombo-occlusive disease","volume":"19","author":"Chun","year":"2005","journal-title":"J Cardiothorac Vasc Anesth"},{"key":"10.1016\/j.rpth.2025.102979_bib76","doi-asserted-by":"crossref","first-page":"2571","DOI":"10.1182\/blood-2003-07-2316","article-title":"Severe hypodysfibrinogenemia in compound heterozygotes of the fibrinogen A\u03b1IVS4 + 1G>T mutation and an A\u03b1Gln328 truncation (fibrinogen Keokuk)","volume":"103","author":"Lefebvre","year":"2004","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib77","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1159\/000205692","article-title":"Multiple pulmonary emboli in a patient with afibrinogenaemia","volume":"79","author":"Cronin","year":"1988","journal-title":"Acta Haematol"},{"key":"10.1016\/j.rpth.2025.102979_bib78","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1002\/ajh.20110","article-title":"Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: a report of two successful pregnancies","volume":"76","author":"Roqu\u00e9","year":"2004","journal-title":"Am J Hematol"},{"key":"10.1016\/j.rpth.2025.102979_bib79","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0290571","article-title":"Differences in coagulation-relevant parameters: comparing cryoprecipitate and a human fibrinogen concentrate","volume":"18","author":"Stanford","year":"2023","journal-title":"PLoS One"},{"key":"10.1016\/j.rpth.2025.102979_bib80","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1111\/jth.14392","article-title":"Clinical pharmacology, efficacy and safety study of a triple-secured fibrinogen concentrate in adults and adolescent patients with congenital fibrinogen deficiency","volume":"17","author":"Djambas Khayat","year":"2019","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib81","article-title":"Are all fibrinogen concentrates the same? The effects of two fibrinogen therapies in an afibrinogenemic patient and in a fibrinogen deficient plasma model. A clinical and laboratory case report","volume":"11","author":"Goodarzi","year":"2024","journal-title":"Front Med (Lausanne)"},{"key":"10.1016\/j.rpth.2025.102979_bib82","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1007\/BF00252836","article-title":"Increased prothrombin activation in a patient with congenital afibrinogenemia is reversible by fibrinogen substitution","volume":"72","author":"Korte","year":"1994","journal-title":"Clin Investig"},{"key":"10.1016\/j.rpth.2025.102979_bib83","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1016\/j.thromres.2011.12.027","article-title":"Novel management of anticoagulant resistant thrombotic hypodysfibrinogenaemia","volume":"130","author":"Rea","year":"2012","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2025.102979_bib84","doi-asserted-by":"crossref","first-page":"e108","DOI":"10.1111\/hae.12584","article-title":"Successful pregnancy under fibrinogen substitution in a woman with congenital afibrinogenaemia complicated by a postpartum venous thrombosis","volume":"21","author":"Lebreton","year":"2015","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib85","doi-asserted-by":"crossref","first-page":"100","DOI":"10.3390\/ijms19010100","article-title":"Identification of two novel fibrinogen B\u03b2 chain mutations in two Slovak families with quantitative fibrinogen disorders","volume":"19","author":"Simurda","year":"2017","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib86","doi-asserted-by":"crossref","first-page":"e431","DOI":"10.1111\/hae.12734","article-title":"Venous thrombosis in afibrinogenemia: a successful use of rivaroxaban","volume":"21","author":"Margaglione","year":"2015","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2025.102979_bib87","doi-asserted-by":"crossref","first-page":"3482","DOI":"10.3390\/jcm13123482","article-title":"Safety of fibrinogen concentrate in non-trauma and non-obstetric adult patients during perioperative care: systematic review and meta-analysis","volume":"13","author":"Gomes","year":"2024","journal-title":"J Clin Med"},{"key":"10.1016\/j.rpth.2025.102979_bib88","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1182\/blood.2024025712","article-title":"How I treat quantitative fibrinogen disorders","volume":"145","author":"Casini","year":"2025","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2025.102979_bib89","first-page":"934","article-title":"Fibrinogen \u0141\u00f3d\u017a: a new cause of dysfibrinogenemia associated with recurrent thromboembolic arterial events","volume":"129","author":"Treli\u0144ski","year":"2019","journal-title":"Pol Arch Intern Med"},{"key":"10.1016\/j.rpth.2025.102979_bib90","article-title":"A quest to find the aetiology of pulmonary embolism beyond the common: a case of dyshypofibrinogenemia presenting as pulmonary embolism","volume":"15","author":"Siddiqui","year":"2023","journal-title":"Cureus"},{"key":"10.1016\/j.rpth.2025.102979_bib91","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1017\/cjn.2018.57","article-title":"Ischemic strokes in a man with congenital afibrinogenemia","volume":"45","author":"Nathoo","year":"2018","journal-title":"Can J Neurol Sci"},{"key":"10.1016\/j.rpth.2025.102979_bib92","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2024.1272802","article-title":"The FGG c.952G>A variant causes congenital dysfibrinogenemia characterized by recurrent cerebral infarction: a case report","volume":"15","author":"Ying","year":"2024","journal-title":"Front Neurol"},{"key":"10.1016\/j.rpth.2025.102979_bib93","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2017-221375","article-title":"Acute renal artery infarction secondary to dysfibrinogenemia","volume":"2017","author":"Keinath","year":"2017","journal-title":"BMJ Case Rep"},{"key":"10.1016\/j.rpth.2025.102979_bib94","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1002\/jcla.21722","article-title":"Comparison of prothrombin time derived from CoaguChek XS and laboratory test according to fibrinogen level","volume":"29","author":"Kim","year":"2015","journal-title":"J Clin Lab Anal"},{"key":"10.1016\/j.rpth.2025.102979_bib95","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","year":"2004","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2025.102979_bib96","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000900","article-title":"Antithrombotic treatment in patients with hemophilia: an EHA-ISTH-EAHAD-ESO clinical practice guidance","volume":"7","author":"Schutgens","year":"2023","journal-title":"Hemasphere"},{"key":"10.1016\/j.rpth.2025.102979_bib97","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1055\/s-0038-1654715","article-title":"The novel protease-activated receptor 1 antagonist vorapaxar as a treatment for thrombosis in afibrinogenemia","volume":"44","author":"Tawfik","year":"2018","journal-title":"Semin Thromb Hemost"}],"container-title":["Research and Practice in Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037925003036?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037925003036?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T14:17:09Z","timestamp":1773843429000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2475037925003036"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7]]},"references-count":97,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,7]]}},"alternative-id":["S2475037925003036"],"URL":"https:\/\/doi.org\/10.1016\/j.rpth.2025.102979","relation":{},"ISSN":["2475-0379"],"issn-type":[{"value":"2475-0379","type":"print"}],"subject":[],"published":{"date-parts":[[2025,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The thrombotic paradox in congenital fibrinogen deficiencies: from pathophysiology to practice","name":"articletitle","label":"Article Title"},{"value":"Research and Practice in Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.rpth.2025.102979","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Author(s). Published by Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.","name":"copyright","label":"Copyright"}],"article-number":"102979"}}