摘要
目的对比核黄素反应性脂质沉积性肌病（RR-LSM）与多发性肌炎（PM）患者的临床特点。方法收集2003-2011年之间北京大学第一医院经过临床病理检查确诊的26例RR．LSM和15例PM患者的临床资料，对比两组患者的临床差异。结果26例RR．LSM患者平均起病年龄为（29±10）岁，就诊时平均病程（8．2±8．0）年。15例PM患者平均起病年龄为（43±15）岁，就诊时平均病程为（2．64±3．7）年。两组发病年龄（P=0．001）和就诊延误时间（P=0．003）差异有统计学意义。两组患者四肢肌肉、吞咽肌、颈肌、呼吸肌无力以及肌痛差异无统计学意义，但RR-LSM患者均存在运动不耐受和肌无力症状波动性。咀嚼困难出现在65．38％的RR-LSM患者和26．67％的PM患者中，两组差异有统计学意义（P=0．017），两组患者均存在骨骼肌外表现，PM更突出。肌酸激酶平均值在RR—LSM组为（1475±1796）U/L，在PM组为（40624-3448）U/L，两组差异有统计学意义（P=0．017）。结论与PM相比，RR-LSM发病年龄小，延误诊断时间长，肌无力具波动性，咀嚼肌受累常见，但骨骼肌外损害少，肌酸激酶升高幅度小。
Objective To compare the clinical characteristics of riboflavin responsive lipid storage myopathy （RR-LSM） versus polymyositis （PM）. Methods The clinical data were collected and analyzed for 26 RR-LSM and 15 PM patients from 2003 to 2011 at Peking University First Hospital. Results The mean onset ages of RR-LSM and PM were （29± 10） and（43 ± 15） years while the durations before definite diagnosis were （ 8.2 ± 8. 0） and （2. 6 ± 3.7） years respectively. There were significant intergroup differences in onset age （ P = O. 001 ） and duration before definite diagnosis （ P = O. 003 ）. No significant inter-group difference existed in the frequency of limb weakness, dysphagia, neck weakness, dyspnea or myalgia. Dysmasesia occurred in 65.38% of RR-LSM and 26. 67% of PM patients and significant inter-group difference existed （P = 0. 017）. All RRLSM patients had exercise intolerance with a fluctuating course. Both groups had extra-muscular symptoms and it was more obvious in PM. Mean creatine kinase （CK） level was（1475 ± 1796）U/L in RR-LSM and （4062 ± 3448）U/L in PM patients with significant inter-group difference （P = 0. 017 ）. Conclusion Comparing with PM patients, RR-LSM patients tend to have an earlier onset, a longer diagnosis delay time, fluctuating weakness, a higher frequency of dysmasesia. But there are fewer extra-muscular svmntoms and a smaller elevation of CK level.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2014年第7期503-506,共4页
National Medical Journal of China
基金
科技部十二五重大专项课题
关键词
核黄素反应性脂质沉积性肌病
多发性肌炎
临床特点
Riboflavin responsive lipid storage myopathy
Polymyositis
Clinical characteristic
