July 1974

Sickle Cell Disease and the Kidney: A Hypothesis

Arch Intern Med. 1974;134(1):181. doi:10.1001/archinte.1974.00320190183032

To the Editor.  —The section concerning sickle cell disease in the review of prostaglandins in hematology1 deserves additional comment.The most common renal abnormality in patients with sickle cell disease is a concentrating defect, associated in many older patients with focal renal papillary necrosis and scarring.The pathogenesis of these functional and anatomic abnormalities is unknown. Perillie and Epstein2 have argued that the hypertonicity present in the renal medulla may lead to erythrocyte sickling in the vasa recta that is independent of oxygen tension, with resultant increased blood viscosity, decreased medullary blood flow, and decreased oxygen delivery to the renal tubular cells. The morphologic studies of Statius van Eps et al3 are consistent with reduced medullary blood flow.An alternative hypothesis is suggested by data recently presented by Johnson et al4 and Rabinowitz et al.5 They observed sickling associated with a marked decrease in oxygen

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