ARTHROPATHY IN CHILDREN WITH SEVERE HEMOPHILIA A

Abstract

Soreff, J. and Blombäck, M. (Departments of Orthopaedic Surgery and Blood Coagulation Disorders, Stockholm, Sweden). Arthropathy in children with severe hemophilia A. Acta Paediatr Scand, 69:667, 1980.—Seven children with severe hemophilia A were studied. The radiological findings show that the patients developed joint changes despite our prophylactic treatment, even if no restriction of movement or deformity could be detected by physical examination. In contrast to earlier reports indicating that changes in the knee joints were most common, our findings show that changes in the ankle joints predominate. Such changes developed, even after less than 10 registered joint bleeding episodes. Prophylactic treatment must start early and should aim to eliminate subclinical bleedings also. Increased dosage and shorter intervals between treatments lead to less bleedings. The dosage must be so high and the time interval so short that the F VIII level in plasma is > 0.01 U/ml at the time of the next infusion. Prophylactic treatment must he regular and should be given even if bleeding requiring treatment with a therapeutic dose occurred in the previous day.