| Título: | Duchenne muscular dystrophy in northeastern Thai children: a retrospective study |
| Autores: |
Narong Auvichayapat Sompon Tassniyom Sarinya Srisarakham Paradee Auvichayapat |
| Fecha: | 2009-12-27 |
| Publicador: | Asian biomedicine: research reviews and news |
| Fuente: |
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| Tipo: | Peer-reviewed Article |
| Tema: | No aplica |
| Descripción: | Background: Duchenne muscular dystrophy (DMD) is the most common hereditary neuromuscular disease. No curative treatment for DMD is known. Prednisone therapy is the first medical treatment that alters the course of DMD. Several studies about the doses and administrations of prednisone or prednisolone had been reported. Objectives: To review clinical features, laboratory findings, and the result of treatment of DMD. Methods: DMD patients who came to Srinagarind Hospital, Thailand from January, 1995 to January, 2007 were retrospectively analyzed. Results: Sixty-two patients fulfilled the study criteria. All patients were male (100 %). Mean age at onset was 4.9 years. Family history was found in 10 families (16 %). The most common symptoms were weakness, standing difficulty, and gait abnormality (100, 97, and 93 % respectively). The most common clinical signs were calf hypertrophy, weakness, and Gower sign (100, 100, and 94 % respectively). Serum creatine kinase (CK) was raised in all of the patients with mean serum CK 13,026 IU/L. Fifty patients received prednisolone. Twelve received only supportive treatments. The overall outcomes of prednisolone treatment were better, same, and worse in 37, 51, and 12 % respectively. Mean age at wheel chair was 10.8 years. Three patients with associate diseases; adult respiratory distress syndrome (ARDS), Sturge Weber syndrome, and autism were presented. To the best of our knowledge, this is the first report about DMD concomitant with ARDS and DMD with Sturge-Weber syndrome. DMD with autism, a very rare occurrence, is presented. Conclusion: Clinical features, laboratory findings, and the outcomes of treatments of 62 DMD patients were presented. Prednisolone treatment had some beneficial effects and had significant side effects. Starting with a low dose, and then increasing to high dose in the no response patient is recommended. |
| Idioma: | No aplica |