The success story of hemophilia care first began in the 1970s, when the availability of
plasma-derived concentrates of coagulation factor VIII (FVIII) and factor IX (FIX) provided …

Fibrinogen is an abundant protein synthesized in the liver, present in human blood plasma
at concentrations ranging from 1.5-4 g/L in healthy individuals with a normal half-life of 3-5 …

Guidelines Management of severe peri-operative bleeding: Guidelines from the European
Society of Anaesthesiology and Intensive Care

The vascular endothelium has several important functions, including hemostasis. The
homeostasis of hemostasis is based on a fine balance between procoagulant and …

Activated factor VII (FVIIa), the first protease of clotting, expresses its physiological
procoagulant potential only after complexing with tissue factor (TF) exposed to blood. Deep …

Introduction The rare coagulation disorders may present significant difficulties in diagnosis
and management. In addition, considerable inter‐individual variation in bleeding phenotype …

Fibrinogen is a complex protein playing a major role in coagulation. Congenital
afibrinogenemia, characterized by the complete absence of fibrinogen, is associated with …

INTRODUCTION von Willebrand disease, hemophilia A, and hemophilia B account for most
inherited coagulation factor deficiencies worldwide. Inherited rare coagulation factor …

Congenital fibrinogen deficiency (CFD) is a rare bleeding disorder caused by mutations in
FGA, FGB, and FGG. We sought to comprehensively characterize patients with CFD using …

Though hundreds of drugs have been approved by the US Food and Drug Administration
(FDA) for treating various rare diseases, most rare diseases still lack FDA-approved …