A case of congenital factor XIII deficiency in a 12-year-old boy is described. The bleeding episode was started with umbilical bleeding and persisted as hemorrhagic disorder associated with slow wound healing.
All usual clotting tests were normal except for a small amplitude and a rapid reduction of amplitude in the thrombelastogram. Diagnosis was established by the clot solubility test, the monoiodoacetate (MIA) tolerance test and the quantitative test with antifactor XIII serum.
In the family history, his parents were cousins, whose plasma factor XIII levels were reduced, and a cousin of maternal grandmother was a bleeder.
The transfusions of Cohn fraction I, fibrinogen and purified factor XIII preparations were evaluated.
