{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:32:38Z","timestamp":1790814758705,"version":"4.1.0"},"reference-count":28,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2022,8,6]],"date-time":"2022-08-06T00:00:00Z","timestamp":1659744000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100016300","name":"Octapharma","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100016300","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Congenital fibrinogen deficiency (CFD) is a rare coagulation disorder placing patients at increased bleeding risk. Human fibrinogen concentrate (HFC) represents current standard of care for fibrinogen replacement in CFD, however, limited data are available on HFC for prophylactic administration before\/during surgery. Here, we report results and dosing considerations for HFC treatment in perioperative bleeding management in adult, adolescent, and pediatric patients with CFD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design and Methods<\/jats:title><jats:p>FORMA\u201002\/FORMA\u201004 were multinational, prospective, open\u2010label, uncontrolled Phase 3 HFC efficacy\/safety studies for surgical bleeding prophylaxis in adult\/adolescent (\u226512\u2009years) and pediatric patients (&lt;12\u2009years) respectively. HFC dosing was calculated to achieve pre\u2010established target fibrinogen plasma levels. Overall hemostatic efficacy was assessed as success\/failure by an Independent Data Monitoring and Endpoint Adjudication Committee (IDMEAC) according to objective criteria.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twelve patients (\u226512\u2009years, <jats:italic>N<\/jats:italic>\u00a0=\u00a09; &lt;12\u2009years, <jats:italic>N<\/jats:italic>\u00a0=\u00a03) received HFC for surgical prophylaxis (15 surgeries; 13 minor, 2 major). Eleven minor surgeries in patients aged \u226512\u2009years required a median of 1 infusion (range; 1\u20135), with a mean (\u00b1SD) dose of 93.50\u2009mg\/kg [\u00b141.43] and two minor surgeries in patients &lt;12\u2009years required 1 infusion (91.55\u2009mg\/kg [\u00b123.40]). The major surgery in an adult patient required eight infusions (225.3\u2009mg\/kg total dose). The major surgery in a pediatric patient required six infusions (450.4\u2009mg\/kg). All surgeries were rated successful by the IDMEAC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>In adults\/adolescents and pediatric patients with fibrinogen deficiency, HFC treatment for hemostatic management during\/after minor and major surgery was successful, with efficacy comparable across the different age groups.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.17029","type":"journal-article","created":{"date-parts":[[2022,8,6]],"date-time":"2022-08-06T10:09:37Z","timestamp":1659780577000},"page":"1871-1881","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Efficacy and safety of fibrinogen concentrate for perioperative prophylaxis of bleeding in adult, adolescent, and pediatric patients with congenital fibrinogen deficiency: <scp>FORMA<\/scp>\u201002 and <scp>FORMA<\/scp>\u201004 clinical trials"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5584-5049","authenticated-orcid":false,"given":"Claudia Djambas","family":"Khayat","sequence":"first","affiliation":[{"name":"Hotel Dieu de France Hospital Saint Joseph University  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunil","family":"Lohade","sequence":"additional","affiliation":[{"name":"Sahyadri Specialty Hospital  Pune India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omid Reza","family":"Zekavat","sequence":"additional","affiliation":[{"name":"Hematology Research Center, Nemazee Hospital Shiraz University of Medical Sciences  Shiraz Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irina","family":"Kruzhkova","sequence":"additional","affiliation":[{"name":"Research &amp; Development Department, Octapharma  Lachen Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Solomon","sequence":"additional","affiliation":[{"name":"Research &amp; Development Department, Octapharma  Lachen Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flora","family":"Peyvandi","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"}]}],"member":"311","published-online":{"date-parts":[[2022,8,6]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-02-0595"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.14216"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01831.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02014.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04424.x"},{"issue":"1","key":"e_1_2_8_7_1","first-page":"23","article-title":"Fibrinogen replacement therapy: a critical review of the literature","volume":"10","author":"Franchini M","year":"2012","journal-title":"Blood Transfus"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13058"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2012.03663.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2010.08208.x"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0040-1710015"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.14392"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14230"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2004.08.003"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.14727"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/vox.12424"},{"key":"e_1_2_8_17_1","unstructured":"FORMA\u201002 Clinical Study Protocol.OctapharmaAG 2014.Prospective open\u2010label uncontrolled phase III study to assess the efficacy and safety of Octafibrin for on\u2010demand treatment of acute bleeding and to prevent bleeding during and after surgery in subjects with congenital fibrinogen deficiency. Available athttps:\/\/clinicaltrials.gov\/ProvidedDocs\/26\/NCT02267226\/Prot_001.pdf. Accessed July 22 2021."},{"key":"e_1_2_8_18_1","unstructured":"FORMA\u201004 Study Protocol.OctapharmaAG 2017.Prospective open\u2010label uncontrolled phase III study to assess the efficacy safety and pharmacokinetics of Octafibrin for on\u2010demand treatment of acute bleeding and to prevent bleeding during and after surgery in paediatric subjects with congenital fibrinogen deficiency. Available athttps:\/\/clinicaltrials.gov\/ProvidedDocs\/84\/NCT02408484\/Prot_001.pdf. Accessed July 22 2021."},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/vox.12624"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.13923"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.3390\/pharmaceutics3010053"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.12998\/wjcc.v7.i17.2450"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1159\/000452864"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01681.x"},{"issue":"3","key":"e_1_2_8_25_1","first-page":"180","article-title":"Inherited dysfibrinogenaemia presenting with portal vein thrombosis","volume":"108","author":"Bandyopadhyay R","year":"2010","journal-title":"J Indian Med Assoc"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2015.06.002"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1993.01420170101015"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.3892\/br.2016.755"},{"key":"e_1_2_8_29_1","unstructured":"U.S. Food and Drug Administration. Octapharma Pharmazeutika Produktionsges.m.b.H. FIBRYGA (Fibrinogen [human]) 2017. Available athttps:\/\/www.fda.gov\/vaccines-blood-biologics\/approved-blood-products\/fibryga. Accessed February 16 2021."}],"container-title":["Transfusion"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/trf.17029","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/trf.17029","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/trf.17029","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T22:11:20Z","timestamp":1692655880000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/trf.17029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,6]]},"references-count":28,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2022,9]]}},"alternative-id":["10.1111\/trf.17029"],"URL":"https:\/\/doi.org\/10.1111\/trf.17029","archive":["Portico"],"relation":{},"ISSN":["0041-1132","1537-2995"],"issn-type":[{"value":"0041-1132","type":"print"},{"value":"1537-2995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,6]]},"assertion":[{"value":"2022-02-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-06-02","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-08-06","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}