马方及类马方综合征脊柱侧凸与青少年特发性脊柱侧凸患者肺功能障碍的差异性比较

Pulmonary function test difference of Marfan syndrome and Marfanoid scoliosis patients with adolescent idiopathic scoliosis patients

目的：比较马方和类马方综合征脊柱侧凸（Marfan syndrome and Marfanoid scoliosis,MMS）患者与青少年特发性脊柱侧凸（adolescent idiopathic scoliosis,AIS）患者肺功能的差异及影响因素。方法：1999年9月~2013年4月我院收治的以胸弯（冠状面）为主的马方及类马方综合征脊柱侧凸患者共85例,其中年龄为11~19岁且临床资料完整的患者共40例（MMS组）,男14例,女26例;收集患者术前肺功能指标,包括第1秒最大呼气容积（FEV1）、用力肺活量（FVC）、最大用力呼气峰流量（PEF）,数值采用实测值占预计值的百分比,分析肺功能指标与年龄、冠状面Cobb角、胸后凸Cobb角、胸弯柔韧度之间的关系。并与同期住院行脊柱侧凸矫形内固定术的相匹配的80例AIS患者（AIS组）的术前肺功能参数进行比较。采用曼-惠特尼U检验比较两组间的差异,并用Pearson相关性分析对两组患者肺功能指标与患者年龄及相关脊柱侧凸指标进行相关性分析。结果：MMS组患者中肺功能处于中、重度损害的比例（11/40）显著高于AIS组（5/80）（P〈0.05）。MMS组患者FEV1、FVC均明显小于AIS组患者（P〈0.05）;PEF两组间差异无统计学意义。MMS组患者FEV1、FVC与冠状面Cobb角呈显著性负相关（r=-0.444、-0.524,P〈0.05）;FEV1、FVC、PEF与年龄之间呈正显著性相关（r=0.363,0.326,0.348,P〈0.05）;FVC与胸弯冠状面柔韧度之间呈显著性正相关（r=0.321,P〈0.05）;FEV1、FVC与胸椎后凸角均无显著相关性。AIS组患者FEV1、FVC、PEF等指标与冠状面Cobb角呈显著性负相关（r=-0.338、-0.293、-0.253,P〈0.05）;FEV1、PEF与年龄之间呈显著性正相关（r=0.286、0.341,P〈0.05）;FEV1与胸后凸Cobb角之间呈显著性正相关（r=0.238,P〈0.05）。两组患者肺功能指标与相关指标间的相关性存在差异。结论：MMS患者肺功能损害较AIS患者严重,其肺功能主要受胸弯冠状面Cobb角、年龄共同影响。

Objectives： To investigate the difference of the pulmonary function test between the Marfan syn-drome and Marfanoid scoliosis（MMS） patients and adolescent idiopathic scoliosis（AIS） patients, as well as the possible risk factors. Methods： In this retrospective study, 40 MMS patients（aged 11-19 years, 14 boys and26 girls） who received posterior correction（group MMS） and fusion and 80 matched AIS patients（group AIS）who received the similar surgery during the same period were included. The essential feature and severity of scoliosis were similar in both groups. The preoperative pulmonary function tests of the two groups were inves-tigated, including forced expiratory volume in one second（FEV1）, forced vital capacity（FVC） and the maximum peak expiratory flow（PEF）, and the risk factors were analyzed. Differences in pulmonary function tests among2 groups were analyzed by using U-test, and Pearsons′ correlation was used to investigate the relationship between pulmonary function and risk factors. Results： Based on the results of pulmonary function tests, the proportion of patients with severe damage in group MMS（11/40） was significantly higher than that of group（5/80）（P〈0.05）. MMS patients had smaller values of FEV1 and FVC than those of AIS patients（P 〈0.05）. No significant difference of PEF was found between the two groups. In group MMS, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes（FEV1 and FVC）（r=-0.444,-0.524, P〈0.05）; the age was positively correlated with the percentage of predicted pulmonary volumes（FEV1,FVC and PEF）（r=0.363, 0.326, 0.348, P〈0.05）; the flexibility of the thoracic curve was positively correlated with the percentage of predicted FVC（r=0.321, P〈0.05）. FEV1 and FVC were not related with thoracic kyphosis. In group AIS, the Cobb angle of thoracic curve was negatively correlated with the percentage of predicted pulmonary volumes（FEV1, FVC and PEF）（r=-0.338,-0.293,-0.253; P〈0.05）. The age was positively correlated with the percentage of predicted pulmonary volumes（FEV1 and PEF）（r=0.286, 0.341; P〈0.05）; the Cobb angle of thoracic kyphosis was positively correlated with the percentage of predicted pulmonary volumes（r=0.238, P〈0.05）. Conclusions： The damage of pulmonary function is more severe in MMS patients than that in AIS patients, and the risk factors that lead to the difference include the age and the Cobb angle of thoracic curve.