A case of congenital afibrinogenemia and review of reported cases in Japan

Congenital afibrinogenemia was first reported in 1920 by Rabe and Salomon. In Japan, since Saito and Asai reported the first case in 1954, 14 cases have been reported so far. The authors found the 10th case and made examinations on clot-ting factors and comparative evaluations on 14 cases reported in Japan. Immunological assay for fibrinogen was performed on 5 out of 14 cases. In our own case, fibrinogen was not demonstrated even by Immunological test. Accelerated fibrinolytic activities are considered to be a cause of afibrinogenemia. Our case is the first one in which remarkably decreased fibrinolvtic activities were demonstrated, and this finding is of interest in relation to deficient clotting ability of blood in this disease.