ACUTE INTRAVASCULAR HEMOLYSIS IN GLUCOSE-6-PHOSPHATE-DEHYDROGENASE DEFICIENCY

Thirty-five children with G6PD deficiency, who presented with acute in travascular haemolysis, were evaluated to define its aetiology, clinic al features and ultimate outcome. All were boys with ages ranging from 6 months to 12 years. Pallor of abrupt onset and passage of cola-colo ured urine were universal presenting symptoms. Incriminating factors r esponsible for haemolysis include hepatitis (7), malaria (4), bacteria l sepsis (3) and drug intake (24), with more than one predisposing con dition existing in some children. Marked elevations in serum bilirubin , coinciding with intravascular haemolysis, was a feature in all the s even children with hepatitis. Azotaemia was noted in 20 patients, of w hom 14 did not have oliguria. All four children with malaria presented with protracted renal failure. Therapy focused on maintaining a high urine output in those without oliguria. A total of 15 peritoneal dialy ses and five haemodialyses were required in six patients with acute re nal failure, all of whom were oliguric. Supportive therapy consisted o f blood transfusions and treatment of the predisposing diseases. Thirt y-two children recovered completely while three died, the cause of dea th being severe anaemia and congestive cardiac failure, malaria with o liguric renal failure and hepatic encephalopathy, respectively.