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进行性肌营养不良42例临床分析 被引量:2

Progressive muscular dystrophy:an analysis of 42 cases
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摘要 目的探讨进行性肌营养不良(PMD)的临床特点及血清肌酸激酶、肌电图、肌肉病理及免疫组织化学检查对其的诊断价值。方法对42例临床诊断为PMD的患儿的临床特征及肌酸激酶、肌电图、病理资料进行分析。结果42例患儿均为男性。39例有较典型的临床症状,3例为因其他疾病进行检查时偶然发现。所有患儿血清肌酸激酶均显著增高,≤4岁和>4岁且≤8岁组的血清肌酸激酶显著高于>8岁组(P值均<0.01)。34例肌电图检查呈肌源性损害,≤4岁组肌电图检查异常的构成比显著低于>4岁且≤8岁和>8岁组(P值分别为0.019 5、0.013 5)。10例患儿行左侧肱二头肌活组织检查,苏木精-伊红染色和免疫组织化学dystrophin染色均显示为Duchenne型进行性肌营养不良。结论PMD患儿中年幼儿的血清肌酸激酶升高更明显,年长儿的肌电图异常更明显,应重视婴幼儿运动发育迟缓,及早进行血清肌酸激酶筛查。 Objective To discuss the clinical characteristics of progressive muscular dystrophy (PMD) and the values of serum creatine kinase, electromyogram, muscular pathology and immunohistochemistry in diagnosis of PMD. Methods The clinical characteristics, serum creatine kinase, electromyogram and pathologic data of 42 children confirmed of PMD were analyzed. Results All the 42 patients were boys; 39 showed typical symptoms and 3 were diagnosed by examinations aimed at other diseases. Serum creatine kinase was increased in all cases, with the increases in patients ≤4 years and 4-8 years significantly higher than that in patients aged 〉8 years (both P〈0. 01). Electromyogram indicated muscular-derived damage in 34 cases. The abnormal rate of electromyogram was significantly lower in children 〈4 years than those in patients aged 4-8 years and 〉8 years (P = 0. 019 5 and 0. 013 5, respectively). Biceps autopsy was carried out in 10 cases, and dystrophin immunohistochemistry examination indicated Duchenne type PMD in all 10 cases. Conclusion Serum creatine kinase is significantly increased in younger patients with PMD, while electromyogram abnormity is more obvious in elder victims. More attention should be paid to delayed motor development and screening of serum creatine kinase should be conducted timely.
出处 《上海医学》 CAS CSCD 北大核心 2009年第12期1092-1094,I0003,共4页 Shanghai Medical Journal
关键词 进行性肌营养不良 肌酸激酶 肌电图 免疫组织化学 Progressive muscular dystrophy Creatine kinase Electromyogram Immunohistochemistry
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