Korean Journal of Nephrology 1989;8(1):148-154.
결정성 경화증에 의한 만성 신부전 1예
윤성노 , 윤영석 , 방병기 , 양우진 , 정수교 , 김형옥
Abstract
Tuberous sclerosis is a rare disease, which occurrs sporadically or hereditarily and is recognized by its neurological and dermatological manifestations. The classical triad is composed of seizure, mental retarda- tion and adenoma sebaseum on face. A 33-year-old woman was hospitalized with end stage renal disease due to renal angiomyolipoma and maintenance hemodialysis was initiated. Biopsy of facial skin revealed adenoma sebaseum. Abdominal CT showed angiomyolipoma in both kidneys and multiple cystic lesions in both adnexae and retroperitoneal space. And the patient suffered from bilateral pneumothorax which resulted from the invasion of tuberous sclerosis to the lung the lung expanded completely. Emergency closed thoracotomy tube drainage was performed. She has been managing with maintenance hemodialysis until now.
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