All issues > Volume 24(11); 1981

Case Report

J Korean Pediatr Soc. 1981;24(11):1100-1105. Published online November 15, 1981.

A Case of tuberous Sclerosis with Polycystic Kidney.

Won Ik Lee, Il Sin Moon, Poong Man Lee, Jae Chung Lee

¹Department of Pediatrics, Catholic Medical College

Abstract

Tuberous sclerosis is an inherited neurocutaneous disorder characterized by seizures, mental ratardation, cutaneous lesions and visceral hamartomas. The usual renal lesion in tuberous sclerosis is the angiomyolipoma which is a hamartomas, but polycystic kidney is relatively rare. We confirmed tuburous sclerosis on the base of the radiological and clinical characterics in 8 years old girl of mental deficiency with four major cutaneous features and multiple bilateral polycystic kidney. We present a case with a brief review of literature.

Keywords :Tuberous sclerosis, Polycystic kidney, Adenoma sebaceum, Shagreen patch.