Studies in sickle-cell anemia: II. Clinical manifestations of sickle-cell anemia in children (an analysis of thirty-seven cases with observation on the use of ACTH and cortisone in two additional cases)

Abstract

Summary Thirty-seven cases of sickle-cell anemia have been reviewed. The disease occurred in twenty-seven males and ten females. The average age of onset of symptoms was 3.5 years while the average age of first admission to the hospital was 4.5 years. Abdominal and joint pains and anorexia were the most frequent symptoms observed in these cases. Respiratory infections, pallor, icterus, splenomegaly, and cardiac murmurs were the most outstanding physical findings. Some of the clinical difficulties encountered in the diagnosis of sickle-cell anemia are discussed. The parameter test of Winsor and Burch has been useful in the differential diagnosis of this disease. The therapy in these cases was essentially supportive, consisting chiefly of blood transfusions and measures to combat respiratory infections. There were five deaths in this series, representing a mortality of 13.5 per cent. The prognosis in sickle-cell anemia has improved since the advent of modern chemotherapy and antibiotics. Two cases in which ACTH and cortisone were successful in relieving joint pains have been reported. ACTH and cortisone give promise of value in the management of hemolytic crises by controlling severe joint and abdominal pains. Should future clinical observations confirm our preliminary impression regarding the value of ACTH and cortisone in the control of hemolytic crises, the questionable use of splenectomy with its attendant mortality may be discontinued in the management of this disease.