Journal of the Korean Pediatric Society 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
Department of Pediatrics, Catholic Medical College
Polycystic Kidney를 동반한 Tuberous Sclerosis의 1례
이원익, 문일신, 이풍만, 이재청
가톨릭의과대학 소아과학교실
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.
Key Words: Tuberous sclerosis, Polycystic kidney, Adenoma sebaceum, Shagreen patch.




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