目的 探讨假肥大型进行性肌营养不良(DMD)患者肺功能受损的特征和肺功能在DMD患者病变程度及干细胞移植治疗康复效果评定中的价值.方法 测定1例DMD患者行异基因骨髓源成体干细胞移植前后的肺通气功能,取其FVC、FEV1、MVV实测值及其占预计值百分率等指标进行比较分析.结果 行异基因骨髓源成体干细胞移植前,患者FVC和FEV1均为1.4 L,MVV为59.5 L;术后3个月分别为2.12 L、2.12 L和118.00L,分别增加了44%、44%和98%.结论 DMD患者的肺功能损害以限制性通气障碍为特征,表现为FVC明显下降,用力呼气无法超过2s,这一独有的鲜明特征的原因为吸气肌肉尤其是膈肌和主要辅助呼气肌腹肌收缩力减弱所致,因此在DMD患者中开展呼吸训练,尤其是针对主要吸气肌--膈肌和主要辅助呼气肌--腹肌收缩力的康复训练是当务之急,而这一点一直未受重视甚至未被注意到,应予正视;由于肺功能对DMD病变程度的判断、治疗康复效果的评定有重要价值,建议将之纳入DMD的常规检查.%Abstract:[Objective]To describe the patterns of lung function abnormality,and to investigate the changes in spirometry before and after allogeneic hematopoietic stem cell transplantation on a Duchenne's muscular dystrophy (DMD)patient.[Methods]Lung function was measured by maximum expiratory flow-volume loops and whole body plethysmography was measured on a 14-year-old DMD patient before and three months after stem cell transplantation.[Results]Luang function test Was characterized by restrictive pattern manifested by lung volume reduction and increased FEV1/FVC due to muscular weakness.Before stem cell transplantation,the FVC,FEV1 and MVV were 1.4L, 1.4 L and 59.5 L,respectively.Three months after stem cell transplantation,the patient's FVC,FEV1 and MVV were significantly increased to 2.12 L,2.12 L and 118.00 L,respectively.[Conclusion]Although genetically modified myoblast transplantation renmins controversial for DMD,a significant change in spirometry was found in this DMD patient after stem cell transplantation.Furthermore,since DMD is characterized by gradually developing muscular weakness,pulmonary physical rehabilitation should be focused on the training of respiratory accessory muscles.
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