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目的研究幼年皮肌炎(JDM)的临床特征、治疗效果以及转归。方法回顾性分析2003年12月-2011年3月在北京儿童医院住院JDM患儿120例,分析其起病情况、临床表现、实验室检查及辅助检查、治疗方法、随访和预后。结果 120例患儿男55例,女65例;发病年龄1~14岁,平均年龄7岁。患儿均有典型的皮损及不同程度的肌肉症状,83例(69%)患儿有内脏受累,最常受累系统为呼吸系统(48%)。所有患儿肌酶增高,肌电图均表现为肌源性损害。120例患儿均采用糖皮质激素治疗,均在早期加用甲氨蝶呤,有肺损害及重症患儿加用环孢素或环磷酰胺。早期治疗效果及远期预后均较好,120例在急性期死亡7例,死于肺部受累并感染致呼吸衰竭5例,并巨噬细胞活化综合征2例。结论 JDM是一种少见疾病,以肌无力和皮肤损害为突出表现,其皮损具有特征性,各脏器功能评估对诊断和判断疾病严重性非常有益;糖皮质激素联合免疫抑制剂治疗JDM安全有效,且预后较好。
Objective To study the clinical features, therapeutic effect and prognosis of juvenile dermatomyositis (JDM). Methods A retrospective analysis of 120 children with JDM admitted to Beijing Children ’s Hospital from December 2003 to March 2011 was performed to analyze the onset, clinical manifestations, laboratory and laboratory tests, treatment methods, follow - up and prognosis. Results 120 cases of children 55 males and 65 females; the age of onset of 1 to 14 years old, with an average age of 7 years. Children had typical lesions and different degrees of muscular symptoms. 83 (69%) children had visceral involvement and the most commonly affected system was respiratory system (48%). All children with increased muscle enzymes, EMG showed myogenic damage. 120 cases of children were treated with glucocorticoid, are added in the early methotrexate, with lung damage and severe cases plus cyclosporine or cyclophosphamide. Early treatment and long-term prognosis are good, 120 cases of acute death in 7 cases, died of lung involvement and infection caused respiratory failure in 5 cases, and macrophage activation syndrome in 2 cases. Conclusions JDM is a rare disease characterized by myasthenia gravis and skin lesions. The lesions are characteristic. The evaluation of organ function is very useful in diagnosing and judging the seriousness of the disease. JDM is safe with glucocorticoid and immunosuppressive agents Effective, and the prognosis is good.