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Picture of the Month

Picture of the Month Abstract Denouement and Discussion Kocher-Debré-Semelaigne Syndrome Manifestations Kocher1 first reported the association of generalized muscular "hypertrophy" and hypothyroidism; Debré and Semelaigne2 subsequently noted the decrease in muscle size after thyroid therapy. Because of generalized muscle enlargement, especially of the calf muscles, patients with this syndrome usually have an athletic appearance.3 The cause of the large muscles is not known and histopathological findings are not consistent.4Other conditions in which muscles may appear enlarged are certain types of muscular dystrophy, hypertrophia muscularum vera, de Lange's extrapyramidal syndrome of muscular hypertrophy, familial periodic paralysis, lipodystrophy, and myotonia congenita. Large muscles are occasionally found in children with primary hypothyroidism and therefore it may be difficult to determine if these patients should be classified as having Kocher-Debré-Semelaigne syndrome, especially since the pathogenesis of the muscle enlargement is not known. Genetics An autosomal recessive mode of inheritance has been suggested. Treatment After treatment with levo-thyroxine decrease References 1. Kocher T: Zur Verhutung des Cretinismus und cretinoider Zustande nach neuen Forschungen . Dtsch Z Chir 34:556-626, 1892.Crossref 2. Debré R, Semelaigne G: Syndrome of diffuse muscular hypertrophy in infants causing athletic appearance: Its connection with congenital myxedema . Am J Dis Child 50:1350-1361, 1935.Crossref 3. Najjar SS, Nachman HS: The Kocher-Debré-Semelaigne Syndrome: Hypothyroidism with muscular "hypertrophy ." J Pediatr 66:901-908, 1965.Crossref 4. Spiro AJ, Hirano A, Beilin R, et al: Cretinismus with muscular hypertrophy (Kocher-Debré-Semelaigne Syndrome): Histochemical ultrastructural study of skeletal muscle . Arch Neurol 23:340-349, 1970.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120350097020
Publisher site
See Article on Publisher Site

Abstract

Abstract Denouement and Discussion Kocher-Debré-Semelaigne Syndrome Manifestations Kocher1 first reported the association of generalized muscular "hypertrophy" and hypothyroidism; Debré and Semelaigne2 subsequently noted the decrease in muscle size after thyroid therapy. Because of generalized muscle enlargement, especially of the calf muscles, patients with this syndrome usually have an athletic appearance.3 The cause of the large muscles is not known and histopathological findings are not consistent.4Other conditions in which muscles may appear enlarged are certain types of muscular dystrophy, hypertrophia muscularum vera, de Lange's extrapyramidal syndrome of muscular hypertrophy, familial periodic paralysis, lipodystrophy, and myotonia congenita. Large muscles are occasionally found in children with primary hypothyroidism and therefore it may be difficult to determine if these patients should be classified as having Kocher-Debré-Semelaigne syndrome, especially since the pathogenesis of the muscle enlargement is not known. Genetics An autosomal recessive mode of inheritance has been suggested. Treatment After treatment with levo-thyroxine decrease References 1. Kocher T: Zur Verhutung des Cretinismus und cretinoider Zustande nach neuen Forschungen . Dtsch Z Chir 34:556-626, 1892.Crossref 2. Debré R, Semelaigne G: Syndrome of diffuse muscular hypertrophy in infants causing athletic appearance: Its connection with congenital myxedema . Am J Dis Child 50:1350-1361, 1935.Crossref 3. Najjar SS, Nachman HS: The Kocher-Debré-Semelaigne Syndrome: Hypothyroidism with muscular "hypertrophy ." J Pediatr 66:901-908, 1965.Crossref 4. Spiro AJ, Hirano A, Beilin R, et al: Cretinismus with muscular hypertrophy (Kocher-Debré-Semelaigne Syndrome): Histochemical ultrastructural study of skeletal muscle . Arch Neurol 23:340-349, 1970.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Oct 1, 1978

References