3组肌营养不良患者肺通气功能损害的比较与康复对策

Lung function impairment and rehabilitation strategy for patients with muscular dystrophy

目的比较Becker型(BMD)和Duchenne型(DMD)肌营养不良(MD)患者肺通气功能受损的共同特点与不同点,并探讨其康复对策。方法测定BMD(A组)、13岁以上长期坚持康复锻炼的DMD(B组)和几乎从不做康复锻炼的DMD(C组)MD患者的肺通气功能并用统计学方法进行比较。结果A组肺通气功能各主要指标占预计值百分率皆在正常范围,分别是:用力肺活量占预计值百分率(FVC%)为(82±15)%,第1秒用力呼气容积占预测值百分率(FEV1%)为(91±18)%,最大通气量占预测值百分率(MVV%)为(98±32)%;B、C两组皆减低,B组分别是:FVC%为(61±13)%,FEV1%为(72±16)%,MVV%为(82±24)%;C组分别是:FVC%为(37±12)%,FEV1%为(43±15)%,MVV%为(52±19)%。3组数据两相比较差异有统计学意义(P<0.05),但FEV1/FVC比较差异无统计学意义(P>0.05),不过均远远高于健康人群的平均值。结论BMD患者虽然肺通气功能各主要指标占预计值百分率皆在正常范围,但他们与DMD患者一样,明显存在FEV1/FVC比例异常增高的限制性通气,且这种比例不协调的限制性通气是MD患者肺通气功能的共同特点;康复锻炼对减缓DMD患者肺通气功能下降的发展趋势有明显帮助,不过不同时配以针对呼吸肌的康复锻炼措施,并不能改善MD患者FEV1/FVC比例不协调的限制性通气;建议在劝MD患者要进行肢体的康复锻炼的同时还要进行针对呼吸肌的康复锻炼,推荐的康复措施有呼吸操、五段锦、吹气球和仰卧起坐运动。

Objective To compare the characteristics of lung function impairment in three groups of patients with becker＇s muscular dystrophy（BMD） and duchenne＇s muscular dystrophy（DMD） and try to approach the rehabilitation strategy. Methods Lung function were measured and compared statistically for three groups of patients. Patients in Group A suffered from BMD. Patients in Group B were those with DMD and insisted on long-term rehabilitation exercise,while patients in Group C were those with DMD and nearly never had rehabilitation exercise. Results Lung function index of Group A were in normal range as the following： FVC% was （82±15）V% ,FEV1% was （91±18）%, MVV% was （98±32）%. Lung function were impared in Group B and Group C. In Group B,FVC% was （61±13）%,FEV1 % was （72±16） % ,MVV% was （82±24）%. In Group C,FVC% was （37±12）%, FEV1 % was （43 ± 15） %, MVV% was （52 ± 19） %. There were significantly different among the three groups in FVC%, FEV1% and MVV% （P〈0.05）, while no significant differences in FEV1/FV（P〉0.05）, although FEV1/FVC of the three groups were higher than the average of normal population. Conclusion Although the main lung function index of BMD patients were in normal range,the abnormally increased FEV1/FVC in both BMD and DMD patients demonstrated that restrictive ventilation disorder was common characteristic in patients with muscular dystrophy. Rehabilitation exercise might be helpful for slowing the lung function deterioration,while rehabilitation exercise specifically aiming at respiratory muscles might be more helpful in that,it could improve the restrictive ventilation disorder in muscular dystrophy patients.