Management of the haemophilic child.

Abstract

Haemophilia is a hereditary and lifelong bleeding disorder which affects males and is transmitted by apparently normal females. Bleeding commonly occurs after trauma, and in the severely affected patient may occur apparently spontaneously. The haemostatic defect is due to total or partial defi- ciency of factor VIII (anti-haemophilic factor, anti-haemophilic globulin, AHG, AHF) and the severity of the bleeding is fairly well correlated with the level of the factor in the blood (Table Complete deficiency of factor VIII is found in severe classical haemophilia and is associated with spontaneous haemorrhages into muscles and joints with crippling. The patient with only partial deficiency of factor VIII is usually spared haemarthroses and may bleed only after injury or surgical operations.