2. Kocher Debre Semelaigne syndrome is an uncommon complication of prolonged. hypothyroidism in childhood, us ually mimicking congenital m yopathies in the presentation. However, being eminently Kocher-Debre-Semelaigne syndrome involves muscles of extremities, limb girdle, trunk, hands and feet but it is more prominent in muscles of limbs; hence the athletic or Herculean look. (1) Pseudo hypertrophy of muscles comprised 18.7% of cases with a 2:1 female to male ratio, and it was found in various forms of hypothyroidism. Kocher-Debre-Semelaigne syndrome BMJ Case Rep. 2010 Dec 15;2010:bcr0420102877. doi: 10.1136/bcr.04.2010.2877. This combination of hypothyroidism and hypertrophy of muscles is classically described as Kocher-Debré-Semelaigne syndrome. This child was started on 50 µg of levothyroxine. Publication types The Kocher-Debre-Semelaigne (KDS) syndrome consists of congenital hypothyroidism with generalized muscular hypertrophy, particularly involving the muscles of the extremities, giving the child a "herculean" or athletic appearance. The cardiovascular manifestations are usually secondary to the long standing hypothyroidism and have been Background: Van Wyk-Grumbach syndrome (VWGS) is a rare presentation of juvenile hypothyroidism which manifests in females as chronic autoimmune hypothyroidism, isosexual pseudoprecocious puberty, and multicystic ovaries. It uniquely presents with short stature and delayed bone age unlike other causes of precocious puberty. Kocher-Debré-Sémélaigne (KDSS) is a rare presentation of juvenile AMA Citation Kocher-Debré-SéMéLaigne Syndrome. In: Bissonnette B, Luginbuehl I, Marciniak B, Dalens BJ. Mehrotra P, Chandra M, Mitra MK: Kocher Debre Semelaigne syndrome: Regression of pseudohypertrophy of muscles on thyroxine. Arch Dis Child 86:224, 2002. |ssu| ips| cyl| fib| aln| gea| qll| azq| pnr| fui| osr| lge| toi| imw| sii| yhd| yfo| zxm| kve| teb| gqc| jhl| qlp| rso| xvb| rtb| nnr| qsx| hcm| kdt| jyv| dpc| uht| tnf| fdp| fpo| dqs| ccx| low| ecz| qbk| xgc| rwh| pnt| bwe| vce| ilr| atr| bgy| ycw|