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July 1976

Salt-Losing Nephropathy in Prune Belly Syndrome: Reversal Following Unilateral Nephrectomy

Author Affiliations

Department of Pediatrics University of Wisconsin 1300 University Ave Madison, WI 53706

Am J Dis Child. 1976;130(7):778-779. doi:10.1001/archpedi.1976.02120080100012

The prune belly syndrome consists of congenital absence of the abdominal musculature, cryptorchidism, and urinary tract abnormalities including hydronephrosis, megacystis, and megaloureter. Survival is primarily determined by the extent of renal dysplasia and associated lower urinary tract obstruction.1 However, obstruction is not a prominent feature in approximately 80% of these patients.2

A salt-losing nephropathy has been described in interstitial nephritis, chronic pyelonephritis, polycystic disease, and, particularly, in medullary cystic disease.3 There has been one previous report of salt-losing nephropathy in the prune belly syndrome in a male infant with severe dysplasia who died at age 7½ months.4 We describe here a male infant with the prune belly syndrome who had a long-term urinary sodium loss of 8 to 12 mEq/kg of body weight daily.

Report of a Case.—A male infant weighing 1,620 gm was born at 32 weeks' gestation with absence of the abdominal

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