| A Case of tuberous Sclerosis with Polycystic Kidney. |
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Won Ik Lee, Il Sin Moon, Poong Man Lee, Jae Chung Lee |
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Department of Pediatrics, Catholic Medical College |
| Polycystic Kidney를 동반한 Tuberous Sclerosis의 1례 |
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이원익, 문일신, 이풍만, 이재청 |
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가톨릭의과대학 소아과학교실 |
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| Abstract |
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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.
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| Key Words:
Tuberous sclerosis, Polycystic kidney, Adenoma sebaceum, Shagreen patch. |
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