Literature analysis of 104 cases of myositis ossificans progressiva reported in China
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摘要:
目的探讨国内进行性骨化性肌炎的发病情况及临床特点，为临床诊疗提供资料。 方法检出1980~2013年进行性骨化性肌炎病例报道70篇（共104例），对进行性骨化性肌炎的年龄及性别分布、先天指趾畸形、首发症状、最易受累部位、诊断治疗方法等进行统计并分析。 结果我国进行性骨化性肌炎男性患病率高于女性，比例为1.36:1。发病年龄为0~38岁，0~4岁年龄段发病率最高。进行性骨化性肌炎呈全身进行性发展，第一指趾畸形的有60例，首发症状多为头颈部肿块，颈部、脊柱最易受累，肿块多由颈部开始逐渐发展至全身各大关节，造成患者的活动能力受限甚至丧失，其诊断主要依靠临床表现与影像学，但疗效确切的治疗方法未见文献报道。 结论进行性骨化性肌炎尚无特殊的有效疗法，需提高对该病的早期诊断，探索系统诊疗方案，延缓疾病的进展，提高患者生活质量。 -
关键词:
- 进行性骨化性肌炎 /
- 进行性骨化性纤维发育不良 /
- 文献分析
Abstract:ObjectiveTo explore the morbidity and clinical characteristics of myositis ossificans progressiva (MOP) in Chinese patients. MethodsWe reviewed 70 reports of a total of 104 MOP cases during 1980 to 2013 in China and analyzed the patients' age and gender distribution, congenital deformities of the toes, initial symptoms, the most frequently affected region, and diagnosis and treatment of the disease. ResultsThe 104 MOP cases reported contained more male patients than female patients (1.36:1). The age of onset ranged from 0 to 30 years, mostly below 4 years. The patients were characterized by a progressive development of systemic myositis ossificans. Sixty of the patients had deformities of the toes. The initial symptoms were frequently head or cervical masses affecting mostly the neck and spinal column with gradual extension to the major joints of the body to cause limited and even loss of activity. The diagnosis of MOP relies mainly on evaluation of the clinical symptoms and radiographic findings, but so far no effective treatment has been available. ConclusionAn early diagnosis and systemic systematic treatments may help delay the progression of the disease and improve the patients' quality of life. -
表 1 各年龄组病例首发年龄、性别分布情况
Table 1. Distribution of gender in patients with different onset ages
Sex Age（year） 0~8 8~16 16~24 24~32 32~40 Female 30 6 3 1 0 Male 37 5 2 1 2 
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表 2 累及部位情况统计
Table 2. Statistics of affected regions
Clinical manifestation n（cases） Positive rate（%） Onset regions Nape 42 40.38 Cephalic region 20 19.23 Aural region 4 3.85 Coxa 8 7.7 Armpit 1 0.96 Lumbus and back 30 28.85 Extremities 15 14.42 Malformation of toes 60 57.7 Affected region Nape 66 63.46 Thorax 5 4.81 Spine 58 55.77 Shoulder 52 50 Elbow 28 26.92 Wrist 10 9.62 Coxa 46 44.23 Lap 22 21.15 Ankle 6 5.77
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表 3 治疗方法统计
Table 3. Statistics of therapies
Treatment n(cases) Complications Operation 23 New heterotypic ossifications emerge on the postoperative incisions and around; Chondroma;Myocardial fibrosis Hormone 14 None Chinese drugs 10 None Physiotherapy 4 None Skin traction 1 None Antibiotics 3 None Aescine sodium 1 None Immunosuppresant 1 None NSAID 4 None Etidronate Disodium 4 None
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