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Congenital fibrinogen disorders: a retrospective clinical and genetic analysis of the Prospective Rare Bleeding Disorders Database.
Mohsenian S, Palla R, Menegatti M, Cairo A, Lecchi A, Casini A, Neerman-Arbez M, Asselta R, Scardo S, Siboni SM, Blatny J, Zapletal O, Schved JF, Giansily-Blaizot M, Halimeh S, Daoud MA, Platokouki H, Pergantou H, Schutgens REG, Van Haaften-Spoor M, Brons P, Laros-van Gorkom B, Van Pinxten E, Borhany M, Fatima N, Mikovic D, Saracevic M, Özdemir GN, Ay Y, Makris M, Lockley C, Mumford A, Harvey A, Austin S, Shapiro A, Williamson A, McGuinn C, Goldberg I, De Moerloose P, Peyvandi F. Mohsenian S, et al. Blood Adv. 2024 Mar 26;8(6):1392-1404. doi: 10.1182/bloodadvances.2023012186. Blood Adv. 2024. PMID: 38286442 Free PMC article.
Congenital fibrinogen deficiency (CFD) is a rare bleeding disorder caused by mutations in FGA, FGB, and FGG. ...Hotspot variants (FGG, p.Arg301Cys/His and FGA, p.Arg35Cys/His) were present in 51% of dysfibrinogenemia. Obstetric complications were commonly …
Congenital fibrinogen deficiency (CFD) is a rare bleeding disorder caused by mutations in FGA, FGB, and FGG. ...Hotspot …
Fibrinogen concentrate--a potential universal hemostatic agent.
Fenger-Eriksen C, Ingerslev J, Sørensen B. Fenger-Eriksen C, et al. Expert Opin Biol Ther. 2009 Oct;9(10):1325-33. doi: 10.1517/14712590903193051. Expert Opin Biol Ther. 2009. PMID: 19645632 Review.
RESULTS/CONCLUSION: Pasteurized fibrinogen concentrate is derived from human plasma. Its half life is 2.7 days in patients with congenital fibrinogen deficiency. For congenital and acquired deficiency in vivo recovery rates vary from 60% to 109%. ...Acquired …
RESULTS/CONCLUSION: Pasteurized fibrinogen concentrate is derived from human plasma. Its half life is 2.7 days in patients with congenita …
Fibrinogen replacement therapy for congenital fibrinogen deficiency.
Bornikova L, Peyvandi F, Allen G, Bernstein J, Manco-Johnson MJ. Bornikova L, et al. J Thromb Haemost. 2011 Sep;9(9):1687-704. doi: 10.1111/j.1538-7836.2011.04424.x. J Thromb Haemost. 2011. PMID: 21711446 Free article. Review.
This review of published studies was conducted to derive data on patients with congenital fibrinogen deficiency (CFD), including dosing of fibrinogen replacement therapy, outcome, and adverse events, either temporally related or distant to fibrinogen replacem …
This review of published studies was conducted to derive data on patients with congenital fibrinogen deficiency (CFD), …
Efficacy and safety of fibrinogen concentrate for on-demand treatment of bleeding and surgical prophylaxis in paediatric patients with congenital fibrinogen deficiency.
Djambas Khayat C, Lohade S, D'Souza F, Shamanur LG, Zekavat OR, Kruzhkova I, Schwartz B, Solomon C, Knaub S, Peyvandi F. Djambas Khayat C, et al. Haemophilia. 2021 Mar;27(2):283-292. doi: 10.1111/hae.14230. Epub 2020 Dec 16. Haemophilia. 2021. PMID: 33326665 Free PMC article.
BACKGROUND: Congenital fibrinogen deficiency (CFD) is a rare, inherited disorder affecting normal blood clotting function, where patients can experience severe and/or frequent bleeding episodes (BEs). ...Two possibly related adverse events occurred, including …
BACKGROUND: Congenital fibrinogen deficiency (CFD) is a rare, inherited disorder affecting normal blood clotting functi …
Pharmacokinetics, clot strength and safety of a new fibrinogen concentrate: randomized comparison with active control in congenital fibrinogen deficiency.
Ross C, Rangarajan S, Karimi M, Toogeh G, Apte S, Lissitchkov T, Acharya S, Manco-Johnson MJ, Srivastava A, Brand B, Schwartz BA, Knaub S, Peyvandi F. Ross C, et al. J Thromb Haemost. 2018 Feb;16(2):253-261. doi: 10.1111/jth.13923. Epub 2018 Jan 22. J Thromb Haemost. 2018. PMID: 29220876 Free article. Clinical Trial.
Conclusions Bioequivalence was not demonstrated for AUC(norm) , clearance and V(ss) . Larger AUC(norm) and slower clearance were observed for the new HFC. Remaining pharmacokinetic parameters reflected bioequivalence to control. ...
Conclusions Bioequivalence was not demonstrated for AUC(norm) , clearance and V(ss) . Larger AUC(norm) and slower clearance were observed …
Clinical pharmacology, efficacy and safety study of a triple-secured fibrinogen concentrate in adults and adolescent patients with congenital fibrinogen deficiency.
Djambas Khayat C, El Khorassani M, Lambert T, Gay V, Barthez-Toullec M, Lamazure J, Bellon A, Henriet C, Bridey F, Négrier C. Djambas Khayat C, et al. J Thromb Haemost. 2019 Apr;17(4):635-644. doi: 10.1111/jth.14392. Epub 2019 Feb 20. J Thromb Haemost. 2019. PMID: 30661302 Free article. Clinical Trial.
Essentials A novel fibrinogen concentrate was evaluated in patients with congenital fibrinogen deficiency. An open-label, phase 2-3 trial studied pharmacology, efficacy, and safety in patients >6 years. ...Patients/Methods Fourteen patients 40 kg received …
Essentials A novel fibrinogen concentrate was evaluated in patients with congenital fibrinogen deficiency. An open-labe …
Rotational thromboelastography for monitoring of fibrinogen concentrate therapy in fibrinogen deficiency.
Kalina U, Stöhr HA, Bickhard H, Knaub S, Siboni SM, Mannucci PM, Peyvandi F. Kalina U, et al. Blood Coagul Fibrinolysis. 2008 Dec;19(8):777-83. doi: 10.1097/MBC.0b013e32830ef90c. Blood Coagul Fibrinolysis. 2008. PMID: 19002044
In dysfibrinogenemia, smaller Clauss assay responses to spiking were observed, whereas the enzyme-linked immunosorbent assay response was variable. ...Clinical investigations using rotational thromboelastography after in-vivo fibrinogen administration to patients with c …
In dysfibrinogenemia, smaller Clauss assay responses to spiking were observed, whereas the enzyme-linked immunosorbent assay response …
Efficacy and tolerability of a pasteurised human fibrinogen concentrate in patients with congenital fibrinogen deficiency.
Kreuz W, Meili E, Peter-Salonen K, Haertel S, Devay J, Krzensk U, Egbring R. Kreuz W, et al. Transfus Apher Sci. 2005 Jun;32(3):247-53. doi: 10.1016/j.transci.2004.08.003. Transfus Apher Sci. 2005. PMID: 15919240
The efficacy and tolerability of a pasteurised human fibrinogen concentrate were assessed in an open, multi-centre, non-controlled retrospective study in patients with congenital fibrinogen deficiency. Haemostatic efficacy was assessed by laboratory investiga …
The efficacy and tolerability of a pasteurised human fibrinogen concentrate were assessed in an open, multi-centre, non-controlled retrospec …