All issues > Volume 25(1); 1982

Case Report

J Korean Pediatr Soc. 1982;25(1):92-96. Published online January 31, 1982.

The Kocher-Debre-Semelaigne Syndrome.

M Y Chung¹, S I Kwon¹, S J Lee¹, D H Kum¹

¹Department of Pediatrics, Korea General Hospital, Seoul, Korea.

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.

Keywords :Kocher-Debre-Semelaigne syndrome;Hypothyroidism;Muscular hypertrophy