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The Kocher-Debre-Semelaigne Syndrome.

Journal of the Korean Pediatric Society 1982;25(1):92-96.
Published online January 31, 1982.
The Kocher-Debre-Semelaigne Syndrome.
M Y Chung, S I Kwon, S J Lee, D H Kum
Department of Pediatrics, Korea General Hospital, Seoul, Korea.
Kocher-Debre-Semelaigne syndrome 1례
정미영, 권순일, 이선자, 금동혁
고려병원 소아과
Abstract
A 4-year old girl with the Kocher-Debre-Semelaigne syndrome, hypothyroidism with generalized muscular hypertrophy is described. Histologic examination of a calf muscle showed musclular hypertrophy with focal eosinophilic degeneration. The stigmas of hypothyroidism and the muscular hypertrophy disappeared promptly and concomitantly following therapy with desiccated thyroid. Similar cases reported in literature are briefly reviewed. The clinical and histologic changes of the muscles in this syndrome are discussed. The pathogenesis of muscular hypertrophy in hypothyroidism remains unknown.
Key Words: Kocher-Debre-Semelaigne syndrome, Hypothyroidism, Muscular hypertrophy


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