Hyposthenuria in sickle cell anemia: a reversible renal defect.

The frequent occurrence of hyposthenuria in patients with sickle cell anemia has been noted since 1928 (1). McCrory, Goren, and Cornfeld (2), and Kunz, Pratt, Mellin, and Cheung (3), established that the urinary concentration defect was not due to a deficiency of the antidiuretic hormone and Zarafonetis, Steiger, Molthan, McMaster, and Colville (4), reported that subjects with the sickle cell trait also had hyposthenuria. It has been suggested that the renal defect has a genetic basis (4); however, the data to be presented demonstrate the correction of the concentration defect in sickle cell anemia patients in whom intravascular sickling was suppressed by transfusions of normal red blood cells.