{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:32:42Z","timestamp":1790814762864,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,3,10]],"date-time":"2023-03-10T00:00:00Z","timestamp":1678406400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The assessment of clinical history is crucial before referring a patient for further laboratory testing. Bleeding assessment tools (BAT) are developed to standardize clinical evaluation. A small number of patients with congenital fibrinogen deficiencies (CFDs) have been evaluated with these tools without definitive results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>We compared the adequacy of the ISTH\u2010BAT and the European network of rare bleeding disorders bleeding score system (EN\u2010RBD\u2010BSS) to identify patients with CFDs. The correlation between the two BATs and fibrinogen levels and patient clinical grade severity was further analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We included 100 Iranian patients with CFDs. Routine coagulation and fibrinogen\u2010specific tests (fibrinogen antigen [Fg:Ag] and activity [Fg:C]) were performed. The ISTH\u2010BAT and EN\u2010RBD\u2010BSS were used to assess the bleeding score (BS) of all patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The ISTH\u2010BAT and EN\u2010RBD\u2010BSS median (range) were 4 (0\u201316) and 2.21 (\u22121.49 to 6.71), with a statistically significant moderate correlation between the two systems (<jats:italic>r<\/jats:italic>\u00a0=\u00a0.597, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001). In patients with quantitative deficiencies (afibrinogenemia and hypofibrinogenemia), the correlation between Fg:C and the ISTH\u2010BAT was moderately negative (<jats:italic>r<\/jats:italic>\u00a0=\u00a0\u2212.4, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001), while the correlation between Fg:C and the EN\u2010RBD\u2010BSS was weakly negative (<jats:italic>r<\/jats:italic>\u00a0=\u00a0\u2212.38, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001). Overall, 70% and 72% of patients with fibrinogen deficiencies were correctly identified by both the ISTH\u2010BAT and EN\u2010RBD\u2010BSS, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These results suggest that in addition to the ISTH\u2010BAT, the EN\u2010RBD\u2010BSS may also be useful in identifying CFD patients. We found a significant level of sensitivity for detecting fibrinogen deficiency in the two BATs, and bleeding severity classification correctly identified severity grades in almost two\u2010thirds of patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14773","type":"journal-article","created":{"date-parts":[[2023,3,10]],"date-time":"2023-03-10T16:07:03Z","timestamp":1678464423000},"page":"827-835","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Diagnostic utility of bleeding assessment tools in congenital fibrinogen deficiencies"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5004-4456","authenticated-orcid":false,"given":"Samin","family":"Mohsenian","sequence":"first","affiliation":[{"name":"Universit\u00e0 degli Studi di Milano, Department of Pathophysiology and Transplantation  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omid","family":"Seidizadeh","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Milano, Department of Pathophysiology and Transplantation  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Palla","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Milano, Department of Pathophysiology and Transplantation  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammad","family":"Jazebi","sequence":"additional","affiliation":[{"name":"Iranian Comprehensive Hemophilia Care Center  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Azita","family":"Azarkeivan","sequence":"additional","affiliation":[{"name":"Blood Transfusion Research Center High Institute for Research and Education in Transfusion Medicine  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Somayeh","family":"Moazezi","sequence":"additional","affiliation":[{"name":"Iranian Comprehensive Hemophilia Care Center  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammad Reza","family":"Baghaipour","sequence":"additional","affiliation":[{"name":"Iranian Comprehensive Hemophilia Care Center  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marzia","family":"Menegatti","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7423-9864","authenticated-orcid":false,"given":"Flora","family":"Peyvandi","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Milano, Department of Pathophysiology and 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