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目的:分析53例儿童皮肌炎(juvenile dermatomyositis,JDM)患者的病史资料,以加强临床对该病的认识。方法:对2001年至2012年的53例病史资料完整的JDM患者进行回顾性分析,内容包括临床表现、实验室检查、治疗方案及近期随访情况。结果:53例患儿中面部水肿性红斑发生率达98%,Gottron丘疹为89%,甲周毛细血管扩张性红斑为30%,钙盐沉积为6%。肌力检测下降者有66%;肌酸激酶同工酶MB(creatine kinase MB,CK-MB)异常升高达70%;肌电图检测率为57%,其中73%者结果异常;肌肉活检检测率为10%,结果均为异常;MRI检测率为42%,检出异常者占78%。所有患者都接受了激素治疗,其中40%加用了丙种球蛋白静脉滴注后好转。24例平均随访7.7年,其中75%的病例症状部分缓解。结论:MRI检查能灵敏而无创地显示出JDM的肌肉病变。丙种球蛋白辅助治疗可减少患者激素的用量,且应用相对安全。JDM病程大多为慢性,需要长期观察。
Objective: To analyze the history data of 53 cases of juvenile dermatomyositis (JDM) in order to enhance the clinical understanding of the disease. Methods: A retrospective analysis was performed on 53 patients with complete history of JDM between 2001 and 2012, including clinical manifestations, laboratory tests, treatment regimens and recent follow-up. Results: The incidence of facial edematous erythema was 98% in 53 children, 89% in Gottron papules, 30% in peritumoral telangiectasia, and 6% in calcium deposits. Muscle test decreased 66%; creatine kinase MB (CK-MB) abnormal increase of up to 70%; electromyography detection rate was 57%, of which 73% of the results were abnormal; muscle biopsy test The rate was 10%, the results were abnormal; MRI detection rate was 42%, 78% were detected abnormalities. All patients received hormone therapy, of which 40% plus gamma globulin infusion improved. Twenty-four patients were followed up for an average of 7.7 years, of which 75% of the cases were partially relieved. Conclusion: MRI can show the muscle lesions of JDM sensitively and noninvasively. Gammolin adjuvant therapy can reduce the amount of patients with hormones, and the application is relatively safe. JDM most of the chronic disease, the need for long-term observation.