首都医科大学学报 ›› 2008, Vol. 29 ›› Issue (6): 766-769.

• 临床研究 • 上一篇    下一篇

儿童进行性骨化性肌炎5例分析

胡冰, 李彩凤, 邝伟英, 王江, 韩彤昕, 何晓琥   

  1. 首都医科大学附属北京儿童医院内科
  • 收稿日期:2008-08-12 修回日期:1900-01-01 出版日期:2008-12-24 发布日期:2008-12-24
  • 通讯作者: 李彩凤

Analysis on Five Pediatric Patients with Myositis Ossificans Progressiva

Hu Bing, Li Caifeng, Kuang Weiying, Wang Jiang, Han Tongxin, He Xiaohu   

  1. Department of Internal Medicine, Beijing Children's Hospital, Capital Medical University
  • Received:2008-08-12 Revised:1900-01-01 Online:2008-12-24 Published:2008-12-24

摘要: 目的 探讨进行性骨化性肌炎的临床特点、影像学表现、临床诊断与治疗方法.方法 回顾性分析5例进行性骨化性肌炎患儿的临床表现、影像学改变、诊断及加重病情的诱因.结果 5例患儿的发病年龄在出生后7d~11岁,特点为反复出现身体局部组织肿块、骨化并伴有邻近关节活动障碍,特征性的指、趾短小畸形以及影像学的相应改变.病情旱进行性加重,外伤、局部刺激可加速病情进展.结论 进行性骨化性肌炎系少见的常染色体显性遗传病,可通过临床表现及特征性指、趾短小畸形进行早期诊断,易误诊而影响疾病进程.目前无特殊治疗方法,重点在于早期诊断及避免加重疾病的诱因.

关键词: 骨化性肌炎, 指/趾, 畸形

Abstract: Objective To investigate the clinical characteristic,image manifestation,diagnosis and the therapeutic methods of the myositis ossificans progressiva(MOP).Methods Five pediatric patients with MOPwere reported.The clinical manifestation,changes in imageology,diagnosis,and the motivation of aggravation were analyzed by using retrospective analysis.The literatures relevant to MOPwere reviewed.Results The average age of onset ranges from 7 to 11 years old in all 5 cases(4 boys and 1 girl).MOP in all the patients were represented as recurrence of local tissue swelling,bone calcification accompanied by disturbance in adjacent joint motion,distinctive malformation of digiti,and corresponding changes in imageology.As the MOPaggravated progressively,trauma and local stimulation could exacerbate the pathogenetic progress.Conclusion MOPis a rare autosomal dominant inherited disease and could be early diagnosed by clinical manifestation and the distinctive malformation of digiti.Misdiagnosis is common and will impact the development of the disease.There has been no special treatment at present and the focus of management should base on early diagnosis as well as avoiding the motivation which might aggravate the disease.

Key words: myositis ossificans, digiti, malformation

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