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A Case of Tuberous Sclelosis with Renal Angiomyolipoma.

Journal of the Korean Pediatric Society 1982;25(11):1170-1174.
Published online November 30, 1982.
A Case of Tuberous Sclelosis with Renal Angiomyolipoma.
H K Park, H J Park, H J Chun, M J Shin, J S Choi
1Dept, of Pediatrics, Seoul Red Croes Hospital, Seoul, Korea
2Dept, of Pathology, College of Medicine, Korea Univ.,Seoul, Korea
신장 혈관근지방종을 동반한 결절성 경화증의 1 례
박형규, 박호진, 전행조, 신미자, 최종상
1서울 적십자병원 소아과
2고려대학교 의과대학 병리학교실
Tuberous sclerosis is a heredofamilial disease and is a multisystem disorder affecting primarily tissues derived from ectoderm, such as skin, eye, central and peripheral nervous system, but also involving organs of mesodermal and endodermal origins, such as heart, lungs, kidneys, bones and GI tract. It is characterized by classical triad of mental retard- ation, epilepsy and angiofibroma. We experienced a full blown case of tuberous sclerosis in 14-year old boy. He has the skin lesions such as angiofibroma, shagreen patch, depigmented nevi and flattened fibroma, and sclerotic bone changes, epilepsy, EEG abnormalities, calcified densities on simple X-ray and "brain CT scan, and mental retradation. And also he has right huge renal angiomyolipoma, which was histogically confirmed. Herein, a case of tuberous sclerosis was presented with a brief review of the related literatures.
Key Words: Tuberous sclerosis, Angiofibroma, Shagreen patch, Depigmented nevi, Angiomyolipoma.

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