DefinePK

Abstract

Introduction


Congenital afibrinogenaemia is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinoge1. All screening tests based on end-point conversion of fibrinogen to fibrin are prolonged and all are corrected by the addition of normal plasma to the patients plasma2. The condition has
been reported in about 150 patients worldwide3. We report two families with congenital afibrinogenaemia, one with 5 out of 6 children being affected and the other with I out of 5 children having the disease.