PARAHEMOPHILIA (OWREN'S DISEASE)

[摘要] A case of parahemophilia in a five year old child has been presented. This case follows the pattern first described by Owren, namely, a congenital hemorrhagic disease with deficient prothrombin activity by the one-stage method correctable by the addition of prothrombin-free plasma. The positive laboratory findings consisted of a prolonged prothrombin time and a slightly prolonged clotting time. Ac globulin deficiency was confirmed both by Dr. Seegers and by Dr. Stefanini. There was no abnormal fibrinolytic activity or fibrinogen deficiency. Serum prothrombin activity indicated that a deficiency of Factor V was accompanied by decreased prothrombin consumption. There was no response to prolonged vitamin K therapy. Blood and plasma transfusions temporarily controlled bleeding, although at no time was a normal one-stage plasma prothrombin time obtained.

[学科分类] 儿科学