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A Clinical Study on Duchenne Muscular Dystrophy

Journal of the Korean Pediatric Society 1992;35(5):652-658.
Published online May 15, 1992.
A Clinical Study on Duchenne Muscular Dystrophy
Moon Sun Yang, Su Ahn Chae, Ki Joong Kim, Yong Seung Hwang
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
Duchenne형 근이영양증에 대한 임상적 고찰
양문선, 채수안, 김기중, 황용승
서울대학교 의과대학 소아과학교실
Abstract
Twenty-five cases of Duchenne muscular dystrophy(DMD) who were diagnosed in the department of pediatrics, Seoul national university hospital (from Jan. 1985 to June 1991) were reviewed. The results were as follows. 1) All of the patients were male. 2) The onset of symptoms was between two and eight years of age. The peak incidence was at four years of age. 3) Chief complaints on admission were walking disturbance (in 16 cases) and abnormally elevated serum transaminase (in 9 cases). 4) The hereditary tendency was confirmed in 9 cases. 5) Both upper and lower extremities were involoved in 8 cases, only lower extremities were involved in 13 cases, and in 3 cases there was no muscle weakness. 6) Mental retardation was noticed in 6 cases and motor developmental delay was noticed in 18 cases. There were no significant correlations between developmental delay and degree of muscle weakness. 7) Serum transaminases, CK and LDH were elevated in all cases. And there were statistically significant inverse correlations between age of patients and the level of serum CK, 8) Changes of EKG were noticed in 17 cases out of 20 cases. 9) Characteristic EMG findings of myopathy were noticed in 18 cases out of 18 cases. 10) Muscle biopsy was performed in all cases and the results were compatible with DMD as a whole.
Key Words: Duchenne muscular dystrophy (DMD), Muscle weakness, Mental retardation, Motor developmental delay, Serum transaminase, Creatin Kinase (CK)


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