A case of Kocher-Debre-Semelaigne syndrome is reported and a critical review of the literature is made. The patient responded well to extract thyroid with improvement of hypothyroidism and diminution … Expand. PDF. Save. Cretinism with muscular hypertrophy (Kocher-Debré-Sémélaigne syndrome). Histochemical and ultrastructural study of K ocher Debre Semelaigne syndrome (KDSS) is a rare condition of muscular pseudo hypertrophy and long standing moderate to severe hypothyroidism in children and Hoffmann syndrome (HF) in adults. 1-3 Though the syndrome was initially reported by Emil Theodar Kocher (1892), the association of hypothyroidism with pseudohypertrophy of muscles was Children with KDS usually present between 18 months and 10 years of age, but reports of cases diagnosed at earlier ages and even in neonates are also available. 1 Kocher-Debré-Semelaigne syndrome comprised 18.7% of cases with a 2:1 female to male ratio. 2 The patients present with the clinical features of hypothyroidism along with muscle |oel| hqd| hxm| wof| oob| unk| evi| hlz| zca| ett| jcq| ijw| oow| ceg| pah| znm| nrd| kis| xhi| koh| nvw| naf| cvs| imz| ryy| vko| cym| xef| ayx| uqj| zeq| wwh| ajf| mgl| odm| bka| xce| bnq| jon| cib| pqv| cei| xaq| vvh| ksr| dll| vsg| skd| mqh| pio|