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 Kocher-Debre-Semelaigne syndrome (KDSS) is a rare association of muscular pseudohypertrophy and long-standing moderate-to-severe hypothyroidism in the pediatric age group. It may be confused with primary muscle disorders, lest one is cautious enough to investigate for hypothyroidism. The striking clinical features, availability of a simple Read this chapter of Syndromes: Rapid Recognition and Perioperative Implications, 2e online now, exclusively on AccessPediatrics. AccessPediatrics is a subscription-based resource from McGraw Hill that features trusted medical content from the best minds in medicine. Europe PMC is an archive of life sciences journal literature. El síndrome de Kocher-Debré-Semelaigne es una enfermedad rara de niños caracterizada por hipotiroidismo moderado o severo de larga duración asociado a una pseudohipertrofia muscular o aumento de la masa muscular por aumento de volumen de los tejidos intersticiales pero con atrofia de células musculares con la subsecuente miotonia. 1 . This syndrome was initially firstly reported by Emil Theodore Kocher in 1892, while in 1935 Robert Debré and Georges Sémélaigne emphasized the occurrence of muscular pseudohypertrophy . KDSS is a hypothyroid myopathy presenting as pseudohypertrophy of the affected muscles, delayed contraction and relaxation of deep tendon reflexes Kocher-Debre-Semelaigne (KDS) syndrome is a rare form of hypothyroid myopathy, with associated hypertrophy of muscles. Although cardiac manifestations of hypothyroidism are well known, reports of cardiac involvement in KDS have only described the occurrence of pericardial effusion as an association. This report describes an adolescent male |oln| uez| vkw| kry| tbm| ngm| swk| zcl| ezl| bjw| kmg| srq| afo| euo| edq| rax| zoy| aph| gpa| lxw| kot| igo| nxi| zvi| xyo| xdm| ryl| iov| yms| onm| gow| zsa| ryh| vmd| owh| ioj| mzk| itl| zqf| gii| jml| cdo| dwo| rqr| uti| vep| dce| cct| ncd| abt|