Abstract Background Factor VII deficiency is a rare, inherited disorder secondary to reduction or absence of factor (FVII) the coagulation pathway presenting with autosomal recessive transmission and caused by different types mutation F7 gene. The clinical manifestations syndrome are highly variable: depending on case, it can remain asymptomatic, present episodes bleeding trauma surgery, manife...

Acquired factor V inhibitor (AFVI) is an extremely rare disorder that may cause severe bleeding. To identify factors associated with bleeding risk in AFVI patients, a national, multicentre, retrospective study was made including all patients followed 21 centres France between 1988 and 2015. All had isolated (FV) deficiency <50% activity. Patients constitutional FV other causes of acquired coagu...

a 28-year-old, 32 week pregnant primigravida woman with a past history of increased blood pressure presented with ruq pain as well as sudden onset of hematemesis. this case illustrates the occurrence of a rare complication (rupture of portal vein pseudoaneurysm inside the biliary system), appearing as upper gastrointestinal bleeding in a pregnant woman. the cause of the rupture is presumably pr...

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. ...

Coagulation factors belong to a family of plasma glycosylated proteins that should be activated for appropriate blood coagulation. Congenital deficiencies of these factors cause inheritable hemorrhagic diseases. Factor VII (FVII) deficiency is a rare bleeding disorder with variable clinical symptoms. Various mutations have been identified throughout the F7 gene and can affect all the protein do...

OBJECTIVE The extent that inherited bleeding disorders affect; number, size and location of bruises in young children <6 years. DESIGN Prospective, longitudinal, observational study. SETTING Community. PATIENTS 105 children with bleeding disorders, were compared with 328 without a bleeding disorder and classified by mobility: premobile (non-rolling/rolling over/sitting), early mobile (cra...

INTRODUCTION The management of patients with coagulopathic disorders undergoing orthopaedic surgery requires a dedicated, multi-disciplinary team with detailed perioperative planning. Bernard-Soulier Syndrome (BSS) is an extremely rare disorder, affecting 1 in 1 million individuals worldwide. It is caused by a deficiency in glycoprotein 1b-V-IX which is required for normal platelet-mediated clo...

Fibrinolysis serves an important role in the process of coagulation, ensuring that clots that are formed in response to injury resolve after the injured tissue is repaired. Fibrinolysis occurs because the protein plasminogen is converted to the active serine protease plasmin by its activating molecules (primarily tissue plasminogen activator). One of the inhibitors of fibrinolysis is alpha(2)-a...

Genetic bleeding disorders form an important presentation among various genetic disorders occurring in children. A prevalence of 6 per 100,000 population has been reported in India. Bleeding disorders constitute a heterogenous group of disorders with varying clinical presentations. The common bleeding disorders are Hemophilia A and B, von Willebrand disease, and inherited thrombocytopenias. In ...