Congenital Coagulation Factor XIII Deficiency Revealed by Convulsion: A Case Report

Factor XIII deficiency is a rare inherited disease, with a particularly high risk of intracerebral hemorrhage. We report the case of a newborn who was suspected to have a coagulation disorder at birth, due to an intracerebral hemorrhage. A quantitative dosage of factor XIII was requested but the usual coagulation tests (thromboplastin, thrombokinase, fibrinogen) were normal. Because of unavailability of specific treatment with factor XIII concentrate, the patient was treated with fresh frozen plasma. The initial dose was for normalizing factor XIII; subsequent monthly doses were designed for preventing the occurrence of serious bleeding.