小儿皮肌炎的临床特征

目的探讨皮肌炎（JDM）患儿的临床特点和疗效。方法回顾性分析1990年1月～2004年1月住本院15例小儿JDM的临床特点,对其血清肌酶、肌肉活检的病理改变及患儿对糖皮质激素的疗效和预后资料进行分析。结果JDM患儿均有对称性近端肌无力;最常见皮肤改变是眼镜状红斑和高春征;患儿均有肌酶升高,肌电图示呈肌源性损害;肌肉活检示肌炎改变,8例经激素治疗1个月肌酶恢复正常,2.5个月肌力改善。结论掌握小儿JDM临床特征对诊断有重要意义,早期诊断、合理治疗可改善预后。

Clinical Features of Juvenile Dermatomyositis

Objective To investigate the clinical manifestations and treatment regiment of children with juvenile dermatomyositis(JDM).Methods The clinical manifestation,changes of serum muscale enzyme,myopathic laboratory examination,treatment and prognosis of 15 children with JDM retrospectively admitted from Jan.1990 to Jan.2004 were analyzed.Results All of the children had symmetrical weakness of the proximal muscles.The most frequent features were heliotrope and Gottron's papules.Elevated muscle enzymes were noted in all cases.Electromyography revealed typical change of myopathic type and muscle biopsy was compalible with myositis in all cases.Most of patients achieved normal muscle enzymes within 1 month and had improved muscle strength with 2.5 monthes of the initiation of corticosteroid therapy.Conclusion It is very important to know the clinical features of JDM,and prompt diagnosis and treatment will result in an improved prognosis.