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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1.4L,MVV为59.5L;术后3个月分别为2.12L、2.12L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。
Objective To investigate the characteristics of pulmonary function impairment in patients with duodenal muscular dystrophy (DMD) and the value of pulmonary function in assessing the severity of DMD and assessing the effect of stem cell transplantation. Methods The lung ventilation function of allogeneic bone marrow-derived adult stem cells in one DMD patient before and after transplantation was measured. The FVC, FEV1, MVV measured values and their percentages of predicted value were compared and analyzed. Results Allogeneic bone marrow-derived adult stem cells before transplantation, the patient FVC and FEV1 were 1.4L, MVV was 59.5L; 3 months after surgery were 2.12L, 2.12L and 118.00L, respectively, an increase of 44%, 44% and 98%. Conclusions Pulmonary dysfunction in patients with DMD is characterized by restrictive ventilatory disturbances, with a marked decrease in FVC and forced expiration of no more than 2 s, a unique and distinctive feature of the inspiratory muscles, especially the diaphragm and the main accessory expiratory muscle Abdominal contractions due to decreased contractility, so in respiratory disease training in patients with DMD, especially for the main inspiratory muscle - diaphragm and the main assistive breathing muscle - abdominal contractility training is a priority, and this one Straight attention has not even been noticed, should be addressed; due to the determination of pulmonary function of DMD lesions, the evaluation of the value of treatment and rehabilitation has important value, it is recommended to be included in the routine examination of DMD.