Which congenital factor deficiency is associated with delayed bleeding after initial hemostasis?
a. Congenital factor XIII deficiency is rare.
b. Factor XIIIor fibrin stabilizing factor is an enzyme (EC 18.104.22.168) of the blood coagulation systemthat crosslinks fibrin. This deficiency leads to defective cross-linking of fibrin and vulnerability to late re-bleeds when the primary hemostaticplug is overwhelmed..
c. It is an recessive disorder Factor XIII deficiency is inherited as autosomal recessive trait.
d. Bleeding is typically delayed because clot form normally but are susceptible to fibrinolysis.
e. Umbilical stump bleeding is characteristic
f. Spontaneous abortion is usual in women with factor XIII deficiency unless they receive replacement therapy.
g. Replacement can be accomplished with FFP, cryoprecipitate or a factor XIII concentrate.
h. Level of 1-2% are usually adequate for hemostasis.
i. With major bleeding or accompanying surgical intervention , the desired concentration of the patient’s plasma is 0.3- 0.5um/mL.
j. With minor bleeding or as a prophylaxis, a level greater than 0.5um/mL is required.