| A Case of Tuberous Sclelosis with Renal Angiomyolipoma. |
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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 |
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박형규, 박호진, 전행조, 신미자, 최종상 |
1서울 적십자병원 소아과
2고려대학교 의과대학 병리학교실 |
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| Abstract |
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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.
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| Key Words:
Tuberous sclerosis, Angiofibroma, Shagreen patch, Depigmented nevi, Angiomyolipoma. |
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