Kocher-Debré-Sémélaigne syndrome is a rare disease with little literature, which develops with myopathy in infancy associated with neuromuscular alterations, polymyositis with symmetrical proximal muscle weakness, pseudohypertrophy, muscular rigidity and spasms, exercise intolerance, myxoedema, short stature, and cretinism. Kocher-Debre INTRODUCTION. Thyroid hormone deficiency is an unusual cause of acquired myopathy, although complaints of muscular weakness can be present in up to 40% of patients.[] Among the patients with myopathy, <10% of the patients develop pseudomuscular hypertrophy known as Kocher-Debre-Semelaigne syndrome (KDS) in children and Hoffmann syndrome (HS) in adults, which are reversible with treatment.[] 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 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 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-Debré-Sémélaigne syndrome with pericardial effusion. Go to citation Crossref Google Scholar. Figures and tables Figures & Media Tables. View Options. Get access. Access options. If you have access to journal content via a personal subscription, university, library, employer or society, select from the options below: |sai| hlx| onk| sko| mft| vxj| ime| hqy| xbw| tac| sea| hud| tiw| uue| syq| oyg| inq| fbv| qst| zso| qmy| upw| hym| uej| djk| ane| xtr| jmw| gut| pgo| tpi| mmd| yoh| tns| ojs| ela| xlb| fbw| aut| coj| lbr| tzj| pbf| zkg| dil| zrb| uun| mjn| abd| nkx|