HYPOSTHENURIA IN SICKLE CELL ANEMIA: A REVERSIBLE RENAL DEFECT

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Abstract
Hyposthenuria was seen in all patients with sickle cell anemia and in 70% of subjects with the sickle cell trait. It is the most common complication associated with the presence of intra vascular S hemoglobin. The ability to concentrate urine was more impaired in older patients with sickle cell anemia than in the younger patients. Following multiple transfusions of normal red cells 4 infants with sickle cell anemia who previously had hyposthenuria were able to concentrate urine normally. The possibility that the urine concentration defect in sickle cell anemia and the sickle cell trait results from renal damage due to either intravascular sickling or from the presence of sickle cell hemoglobin in renal tubular cells is discussed.