Report of Two Cases of Kocher-Debre Semelaigne Syndrome

Yıl 2005, Cilt: 7 Sayı: 3, 25 - 27, 01.12.2005

Hakan Uzun Şeref Targan Özlem Çorumlu Alev Aktaş Füsun Atlıhan

Öz

Myopathy associated with hypothyroidism classically presents with proximal weakness, fatigue, exertional pain, slowed movement, myoedema, diminished deep tendon reflexes, stiffness, myalgia, less commonly cramps and rarely muscle enlargement. Muscle enlargement and hypothroidism is known as Kocher-Debre Semelaigne syndrome (KDS). We report ed here two brothers at the ages of 11 and 7 year-old diagnosed as KDS syndrome. We aimed to emphasize the effects of hypothyroidism on muscle tissue

Anahtar Kelimeler

hypothyroidism, myopathy, child

Kocher-Debre Semelaigne Sendromlu İki Olgu Sunumu

Öz

Hipotroidi ile ilişkili miyopati klasik olarak, proksimal kas güçsüzlüğü, yorgunluk, egzersiz ağrısı, hareketlerde yavaşlama, kas ödemi, derin tendon reflekslerinde azalma, kaslarda sertleşme, ağrı daha nadir olarakta kramp ve kasın büyümesi şeklinde kendini gösterir. Kasın büyümesi Kocher-Debre Semelaigne sendromu (KDS) olarak bilinir. Biz burada 11 ve 7 yaşlarında KDS sendromlu iki erkek kardeş olguyu sunarak hipotroidinin kas dokusu üzerindeki etkilerini vurgulamayı amaçladık

Anahtar Kelimeler

Hipotiroidi, miyopati, çocuk

Kaynakça

• 1. Kung AWC, Ma JTC, Yu YL: Myopathy in acute hypothyroidism. Post Med j 63:661- 663,1987.
• 2. Lochmüller H, Reimers CD, Fischer P: Exercise induced myalgia in hypothyroidism. Clin Invest. 17: 1697-1700,1993.
• 3. Mehrtra P, Chandra M, Mitra MK: Kocher Debre Semelaigne syndrome: regression of pseudohypertrophy of muscles on thyroxine. Arch Dis Child. 86: 224,2002.
• 4. Klein I, Levey G: Unusual manifestations of hypothyroidism. Arch Intern Med. 144:123- 128,1984.
• 5. Klein I, Mantell P, Parker M, Levey GS: Resolution of abnormal muscle enzyme studies in hypothroidism. Am J Med Sci. 279:159-162,1980.
• 6. Thasko V, Davachi F, Baboci R, Drishti G, Hoxha P: Kocher-Debre Semelaigne syndrome. Clin Pediatr (Phila). 38:113- 115,1999.
• 7. Monzani F, Caraccio N, Siciliano G, Manca L, Murri L, Ferrannini E. Clinical and biochemical features of muscle dysfunction in subclinical hypothyroidism. J Clin Endocrinol Metab. 82: 3315-3318,1997.
• 8. Siciliano G, Monzani F, Manca ML, Tessa A, Caraccio N, Tozzi G, Piemonte F, Mancuso M, Santorelli FM, Ferrannini E, Murri L: Human mitochondrial transcription factor A reduction and mitochondrial dysfunction in Hashimoto's hypothyroid myopathy. Mol Med 8: 326- 333,2002.
• 9. Ghilardi G, Gonvers JJ, So A: Hypothyroid myopathy as a complication of interferon alpha therapy for chronic hepatitis C virus infection. Br J Rheumatol. 37:1349-1351,1998.
• 10. Mastropasqua M, Spagna G, Baldini V, Tudesco I, Paggi A: Hoffman’s syndrome: muscle stiffness, pseudohypertrophy and hypothyroidism. Horm Res. 59:105-108,2003.
• 11. Ruurd FD, Bosch J, Laman DM: Neuromuscular findings in thyroid dysfunction aprospective clinical andelectrodiagnostic study. J Neurol Neurosurg Psychiatry. 68:750-755,2000.
• 12. Najjar SS: Muscular hypertrophy in hypothyroid children: The Kocher-Debre-Semelaigne Syndrome. J Ped 85:236-239,1974.