Results of an international, multicentre pharmacokinetic trial in congenital fibrinogen deficiency

Thrombosis Research
Flora Peyvandi

Abstract

Rare bleeding disorders represent 3-5% of all inherited coagulation factor disorders. Inherited afibrinogenemia, which is caused by a deficiency in plasma fibrinogen (coagulation factor I), accounts for only a small portion of all the rare bleeding disorders. Patients with afibrinogenemia have a variable bleeding pattern that may include potentially serious or life-threatening haemorrhages. Treatment of afibrinogenemia is aimed at replacing the missing fibrinogen to restore efficient haemostasis and to stop bleeding. Three sources of fibrinogen are currently available: fresh frozen plasma, cryoprecipitate, and lyophilized fibrinogen concentrate. Owing to its rarity, little is known about the optimal treatment of patients with afibrinogenemia, including the pharmacokinetics of fibrinogen concentrate. To explore this further, we conducted a prospective, open-label, uncontrolled, multinational pharmacokinetic trial of pasteurized human fibrinogen concentrate in 15 patients with afibrinogenemia. Infusion of a single dose of fibrinogen concentrate (70 mg/kg body weight) resulted in similar fibrinogen antigen and activity levels, which were highly correlated. Fibrinogen levels rose rapidly following infusion to reach a maximum of app...Continue Reading

References

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Citations

Mar 31, 2015·Journal of Veterinary Internal Medicine·M A McMichaelS A Smith
Oct 4, 2012·Topics in Companion Animal Medicine·Armelle de Laforcade
Dec 30, 2014·Haemophilia : the Official Journal of the World Federation of Hemophilia·A N ZiaM U Callaghan
Jun 14, 2016·Expert Review of Hematology·Lucia StanciakovaJan Stasko
Aug 9, 2012·Current Opinion in Hematology·Loan Hsieh, Diane Nugent
Jan 1, 2016·Terapevticheskiĭ arkhiv·E V YakovlevaN I Zozulya
May 2, 2017·European Journal of Anaesthesiology·Sibylle A Kozek-LangeneckerKai Zacharowski
Sep 6, 2020·Journal of Thrombosis and Haemostasis : JTH·Fernando F Corrales-MedinaGuillermo De Angulo

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