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平山病是一种青春期起病、以上肢局限肌肉萎缩为主要特征的疾病,多于生长发育高峰时期起病,好发于亚洲男性.由于起病及进展隐匿,早期临床表现与运动神经元病极为相似,因此,平山病早期确诊率较低,患者往往无法得及时有效的治疗.尽管平山病预后明显优于运动神经元病,但缺乏及时的有效治疗,也同样会造成患者出现严重的肌肉萎缩,进而严重影响日常生活和工作.因而需要理论依据充分、涵盖面广、技术指导性强且适用于平山病患者的诊疗指南.基于上述原因,复旦大学附属华山医院骨科、北京大学第三医院骨科和《中华骨科杂志》编辑部组织全世界6个国家、7个专业领域的47位专家遵循科学性、实用性和先进性原则,采用Delphi法共同讨论制定了平山病临床诊疗规范国际指南,从流行病学、临床表现、影像学及电生理特征、鉴别诊断、临床分型、保守与手术治疗、疗效评价等诸多方面系统介绍平山病的诊疗措施,从而规范和推进平山病诊疗发展,提高平山病的早期确诊和有效干预水平.“,”Hirayama disease(HD)is a clinical disease characterized by puberty onset and limited muscle atrophy of the upper extremities.HD usually occurs in Asian males at the peak of growing development.Because of concealed progress of HD and its early clinical manifestations similar to motor neuron disease,the early diagnosis rate of HD is low,therefore,the patients with HD often cannot get timely and effective treatment.Although the prognosis of HD is obviously better than that of motor neuron disease,the lack of timely and effective treatment will cause severe muscle atrophy.Therefore,HD needs a guideline with sufficient theoretical basis,wide coverage,strong technical guidance and suitable for patients with HD.Based on these reasons,forty-seven experts from 6 countries and 7 professional fields around the world were organized by Department of Orthopeadics,Huashan Hospital,Fudan University;Department of Orthopaedics,Peking University Third Hospital;and Editorial Office of Chinese Journal of Orthopaedics;and they discussed and formulated the guidelines that systematically introduces the diagnosis and treatment measures of HD from the aspects of epidemiology,clinical manifestation,imaging and electrophysiological characteristics,differential diagnosis,clinical classification,conservative and surgical treatment,and curative effect evaluation,in order to standardize and promote the diagnosis and treatment of HD and improve the early diagnosis and effective intervention of HD.