Renal abnormalities in patients with sickle cell disease are common. They include a tubular defect resulting in abnormality of concentrating ability1,2; unilateral, intermittent hematuria3 and occasionally, infarctions of the renal parenchyma,1 especially the medulla.2,4 The nephrotic syndrome has also been reported to occur in patients with sickle cell anemia.5-8 Whether this is a fortuitous association or related to the hemoglobinopathy still remains unsettled. Because patients with sickle cell anemia characteristically develop thrombotic and embolic crises,2 it is tempting to implicate thrombosis of the renal venous system in the pathogenesis of the nephrotic syndrome.
In support of this association, the present communication deals with the nephrotic syndrome in a patient with sickle cell disease and demonstrated renal vein thrombosis. Renal function studies performed before and after renal vein recanalization tend to confirm the causal role of the venous occlusion in the development of the nephrotic
Strom T, Muehrcke RC, Smith RD. Sickle Cell Anemia With the Nephrotic Syndrome and Renal Vein Obstruction. Arch Intern Med. 1972;129(1):104–108. doi:https://doi.org/10.1001/archinte.1972.00320010108014
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: