Kocher-Debre-Semelaigne Syndrome: Response to Thyroxine Replacement Therapy
Journal Title: International Journal of Clinical Endocrinology and Metabolism - Year 2016, Vol 2, Issue 1
Abstract
Introduction: Congenital hypothyroidism with muscular pseudohypertrophy or Kocher-Debre-Semelaigne syndrome is the result of long standing untreated moderate to severe hypothyroidism. The pathogenesis of this muscular pseudohypertrophy is unknown and it is usually noted in the muscles of the extremities, limb girdle, trunk, hand and feet but is most evident in the muscles of the lower limb giving the child a Herculean appearance. This is in contrast to the thyroid myopathy due to hypothyroidism in older age group patients which results in muscle atrophy and wasting.
Authors and Affiliations
Tewari Vishal V, Mehta Ritu
Keywords
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- EP ID EP542022
- DOI 10.17352/ijcem.000014
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