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Clinical and Histologic Study of Progressive Muscular Dystrophy Which was Considered as Chronic Hepatitis

Journal of the Korean Pediatric Society 1995;38(2):216-222.
Published online February 15, 1995.
Clinical and Histologic Study of Progressive Muscular Dystrophy Which was Considered as Chronic Hepatitis
Jae Kwang Kim, Ki Sup Chung, Tae Seung Kim
Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
만성 간염으로 오인된 진행성 근이영양증에 대한 임상적 및 조직학적 연구
김재광, 정기섭, 김태승
연세대학교 의과대학 소아과학교실
Abstract
Purpose
: The authors have experienced thirty three children of progressive muscular dystrophy. 10 children of these(30.3%) were considered as chronic hepatitis because of persistent, u nexplained elevated serum aminotransferase values. We performed this study in order to evaluate the characteristics of age distribution, clinical and histologic features in children considered as chronic hepatitis.
Methods
: This study was performed on thirty three children of progressive muscular dystrophy from Febrary, 1983 to June, 1992. 10 children of these(30.3%) were considered as chronic hepatitis because of persistent, unexplained elevated serum aminotransferase values. These 10 children were referred to our pediatric gastroenterologic department for studies of liver dise-ases. Hepatitis viral marker studies were negative in all of them. We performed serum CK concentration, the most useful marker of progressive muscular dystrophy, then confirmation of diagnosis was made by EMG and muscle biopsy in most of them.
Results
: 1) 10 children (30.3%) were considered as chronic hepatitis. 2) 6 cases of 7 (85.7%) before 3 years of age were considered as chronic hepatitis 3) All of them were absence of clinical symptom and sign on admission but later recognized Gowers?sign and muscle weakness in 2 cases over 10 years of age after detailed physical examination. 4) We performed muscle biopsy in 6 cases of 10 children considered as chronic hepatitis. 5 cases were Duchenne muscular dystrophy and 1 cases was Becker muscular dystrophy.
Conclusion
: We concluded that pediatrician need to consider progressive muscular dystrophy when faced with persistent, unexplained serum aminotransferase elevation. In this situations, measurement of serum CK concentration and careful physical examination are the most important and useful means of correctly identifying progressive muscular dystrophy.
Key Words: Progressive muscular dystrophy, Chronic hepatitis, CK


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