inhibitor development is a lifelong challenge for patients with bleeding disorders who received replacement therapy. most commonly, inhibitor formation was observed in hemophilia a patients but patients with rare bleeding disorders (rbd) especially patients with deficiency of factor xiii (fxiii) and factor v (fv) can develop an inhibitor against exogenous factors. several factors considered as ...

factor v (fv) deficiency is a rare bleeding disorder (rbd) that inherit in autosomal recessive manner. diagnosis of fv deficiency (fvd) is made by routine coagulation tests, fv activity and molecular analysis. in patients with fvd, routine coagulation tests including activated partial thromboplastin time (aptt), prothrombin time (pt) and evenbleeding time (bt) are prolongedwhile thrombin time (...

conclusions an hb-driven algorithm, in combination with the “coagulation box” and the early use of clotting factors, could be a simple and effective tool for improving coagulopathy in multiple trauma patients. results over a 12-month investigation period, 123 severe multiple trauma patients needing intensive care therapy were admitted to our trauma center (mean age 48 years, mean iss (injury se...

rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...

Children with symptoms of bleeding and bruising are commonly seen in clinical practice. Primary care providers should be able to decide when and whether evaluation for bleeding disorder is warranted. This decision depends on one's index of suspicion for bleeding disorder based on history, physical examination, and screening laboratory investigations. Knowledge of the hemostatic physiology is es...

congenital factor x deficiency is a rare autosomal recessive bleeding disorder that presents with variable bleeding tendency and prolonged coagulation tests, prothrombin time, and partial thromboplastin time. thromboembolic events have not been reported in patients with factor x deficiency yet. herein, we report a patient with factor x deficiency who had recurrent venous thromboembolic events.

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...

conclusions the case was fully investigated and diagnosed as a case with factor xiii deficiency causing such uncontrolled bleeding after extraction of a tooth. introduction factor xiii deficiency is a relatively rare hereditary bleeding disorder, which is usually found during infancy or early childhood. this condition may involve both genders within different races in an equal manner. its incid...

Hemophilia is a rare X-linkated bleeding disorder which causes spontaneous or secondary bleeding. This used to be treated with plasma cryoprecipitate, but since clotting factor precipitates became available these have become standard of treatment.