Renal Papillary Necrosis Unmasking Sickle Cell Disease

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     A 26-year-old healthy Greek woman was evaluated because of new-onset painless hematuria. The physical examination was unremarkable. Laboratory data were notable for a hemoglobin concentration of 7.6 g per deciliter and a serum creatinine level of 0.7 mg per deciliter (61.9 μmol per liter ). Pyelography that was performed with the use of contrast material revealed bilateral deformities of the renal papillae, including hooks, spurs, and ring-shaped filling defects (sloughed papilla) of the calyxes (arrows), suggestive of papillary necrosis. Interstitial nephritides, such as pyelonephritis and nephritis due to obstructive uropathy, diabetes mellitus, or analgesic abuse, that are associated with papillary necrosis were ruled out. Evaluation of a blood specimen revealed sporadic sickle cells. Electrophoresis of the hemoglobin established the diagnosis of sickle cell–+ thalassemia. The patient's hematuria resolved after bed rest, vigorous hydration with hypotonic fluids, transfusions, and alkalization of the urine. Papillary necrosis due to ischemic damage to the papillae is a well-known complication of sickle cell syndromes but is uncommon at presentation. Sickle cell disease should be considered in patients with unexplained hematuria.

    Michalis Voulgarelis, M.D., Ph.D.

    Panayiotis D. Ziakas, M.D., Ph.D.

    National University of Athens Medical School

    11527 Athens, Greece

    http://www.100md.com/html/DirDu/2007/01/01/32/52/95.htm