Which congenital factor deficiency is associated with delayed bleeding after initial hemostasis?
A. Factor VIICongenital factor XIII (FXIII) deficiency, originally recognized by Duckert in 1960, is a rare autosomal recessive disease usually associated with a severe bleeding diathesis. The male-to-female ratio is 1:1. Although acquired F XIII deficiency has been described in association with hepatic failure, inflammatory bowel disease, and myeloid leukemia, the only significant association with bleeding in children is the inherited deficiency. Bleeding is typically delayed because clots form normally but are susceptible to fibrinolysis. Umbilical stump bleeding is characteristic, and there is a high risk of intracranial bleeding. Spontaneous abortion is usual in women with F XIII deficiency unless they receive replacement therapy. Replacement can be accomplished with fresh frozen plasma(FFP), cryoprecipitate, or a F XIII concentrate. Levels of 1 to 2% are usually adequate for hemostasis.