Congenital Hypoproconvertinemia.

Abstract

Two patients with congenital hemorrhagic disease associated with prolonged Quick test are presented. Laboratory examination showed that this prolongation was not due to deficiency of prothrombin, proaccelerin, or fibrinogen, or to excess of inhibitors. The diagnosis of hypoproconvertinemia was established by findings of a low "proconvertin index," low proconvertin by Owren''s assay technique, and by the ability of added proconvertin to restore normal prothrombin conversion in vitro. Prothrombin consumption tests in both patients showed a moderate decrease in utilization of prothrombin during spontaneous coagulation.