Published June 1, 2009 | Version v1
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"Kocher Debre Semelaigne Syndrome" – A Case Report

  • 1. Department of Pediactrics, *Department of Medicine, People's College of Medical Sciences & Research Centre, People's Campus Bhanpur, Bhopal-462037 (M.P.)

Description

We report a case of 12 year old male child with Kocher-Debré-Semelaigne syndrome (KDSS) which is a rare clinical
presentation of hypothyroidism associated with muscular pseudo-hypertrophy.  The main differential diagnosis is Duchenne
Muscular Dystrophy.  Pseudomuscular hypertrophy of KDSS is an acquired type of myopathy associated with severe and
long standing hypothyroidism and is reversible with thyroxine supplement.

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References

  • 1. Dharaskar P, Tullu MS, Lahiri KR, Kondekar S, Vaswani RK: Kocher-Debre-Semelaigne syndrome with pericardial effusion. Indian Journal of Medical Sciences, 2007; 61(9): 527-530. 2. Ghosh JB: Kocher Debre Semelaigne syndrome in a neonate. Indian Pediatrics, 1988;25(10):1018-1019. 3. Kung A WC, Ma J T C, Yu Y L, Wang C C L, Woo E K W, Lam K S L, Huang C Y, Yeung R T T: Myopathy in acute hypothyroidism. Postgraduate Medical Journal, 1987; 63: 661-663. 4. La Franchi S: Disorders of the thyroid gland. In: Nelson Text book of Pediatrics. RE Behrman , RM Kliegman, HB Jenson (Eds), 17 th Edn;WB Saunders Company, Philadelphia, 2004; pp.1696-1714. 5. Mehrotra P, Chandra M, Mitra MK: Kocher Debre Semelaigne Syndrome. Regression of pseudo- hypertrophy of muscle on thyroxine. Archives of Disease in Childhood, 2002;86(3):224.
  • 6. Tashko V, Davachi F, Baboci R, Drishti G, Hoxha P: Kocher- Debre Semelaigne syndrome. Clinical Pediatrics (Phila), 1999; 38: 113-115. 7. Tullu MS, Udgirkar VS, Muranjan MN, Sathe SA, Kamat JR:Kocher-Debre-Semelaigne syndrome : hypo- thyroidism with muscle pseudo-hypertrophy. Indian Journal Pediatrics, 2003;70:671-367. 8. Yeshwanth M, Benakappa DG, Gautham KS: Kocher Debre Semelaigne Syndrome. Indian Pediatric, 1987; 24(4): 346- 348.