Studies in sickle-cell anemia. XIV. Management of the child with sickle-cell anemia.

Abstract

Sickle-cell anemia (SCA) is a systemic disease characterized by protean symptomatology and by periods of remission and exacerbation. The clinical manifestations are primarily dependent upon hemolysis, stasis, anemia, the age of the patient, and the nature of the precipitating or stressor agent such as infections. The clinical expression of disease often appears in the form of pain, fever, anorexia, debility, weakness, vomiting, pallor, lymphadenopathy, jaundice, impaired renal function (hyposthenuria), leukocytosis, and organ enlargement—particularly involving the heart, liver, and spleen. In infants and small children swelling of the small joints of the hands and feet is a fairly common occurrence. The most commonly encountered hemoglobinopathies in which sickling is of clinical significance in the pediatric age period are (1) homozygous S sickle-cell anemia, (2) sickle-cell-hemoglobin-C disease, and (3) sickle-cell thalassemia (microdepanocytosis). In our experience, 1 sickle-cell-hemoglobin-C disease, in the absence of severe osseous involvement (such as aseptic necrosis) is relatively mild