I型神经纤维瘤病合并脊柱侧凸患者肺功能损害模式的研究  

The pulmonary dysfunction patterns in scoliosis associated with type I

作　　者：邵翔[1] 邱勇[1] 朱锋[1] 朱泽章[1] 孙旭[1] 黄爱兵[1] 蒋军[1] 毛赛虎[1] 

机构地区：[1]南京大学医学院附属鼓楼医院骨科,210008

出　　处：《中华外科杂志》2010年第7期518-521,共4页Chinese Journal of Surgery

基　　金：国家自然科学基金资助项目（30672131）

摘　　要：目的分析I型神经纤维瘤病（NF1）合并脊柱侧凸患者肺功能损害的模式，以及影响其肺功能的影像学因素。方法选取2003年1月至2009年6月间收治的NFl合并脊柱侧凸患者36例（NF1组），特发性脊柱侧凸（IS）患者64例（IS组），术前检测患者的肺活量（VC）、用力肺活量（FVC）、第1秒用力呼气容积（FEV1）、最大呼气中期流量（MMEF）、最大自主通气量（MVV）。比较两组肺功能参数的差异。按照弯型部位及有无萎缩性改变将NF1组患者分类并分类比较肺功能损害的差异，分析影响肺功能的影像学指标。结果两组肺功能参数VC、FVC、FEV1、MMEF、MVV差异均无统计学意义（P〉0．05）。NF1组胸弯患者肺功能显著低于非胸弯患者；营养不良型患者与非营养不良型患者肺功能损害差异无统计学意义（P〉0．05）；顶椎位置以及Cobb角与肺功能参数显著相关（P〈0．05）。结论NF1合并脊柱侧凸患者的肺功能损害模式与IS患者类似，胸弯患者比非胸弯患者肺功能损害严重，侧凸位置以及Cobb角大小是影响患者肺功能的主要因素。Objective To investigate the pulmonary dysfunction patterns in patients of scoliosis associated with neurofibromatosis type I （ NF1 ） and to identify factors affecting the pulmonary function in patients with scoliosis associated with NF1. Methods Preoperative pulmonary function tests （PFrs） were evaluated in 100 patients with scoliosis [ NF1 group, 36 cases; idiopathic scoliosis（IS） group, 64 cases] from January 2003 to June 2009. According to location of apical vertebra and dystrophic change in patients with NF1, the parameters of pulmonary function [ vital capacity （ VC ）, forced vital capacity （ FVC ）, forced expiratory volume in one second （ FEV1 ）, maximal mid-expiratory flow （ MMEF ）, maximal voluntary ventilation（MVV） ] were compared between NF1 group and IS group, and between the subgroups of NF1. The correlation between pulmonary function parameters and radiographic parameters of scoliosis was analyzed. Results The VC, FVC, FEV1, MMEF, MVV in NF1 group and IS group were of no significant difference （P 〉 0. 05）. In NF1 patients, the pulmonary dysfunction was more severe in thoracic subgroup than non-thoracic subgroup （ P 〈 0. 05 ）, while there was no difference between dystrophic scoliosis and non- dystrophic scoliosis （ P 〉 0. 05 ）. The location of apical vertebra and the severity of scoliosis correlated significantly with the pulmonary dysfunction in NF1 group. Conclusions The pattern of pulmonary dysfunction in seoliosis associated with NF1 is similar with IS. Pulmonary dysfunction is more severe in thoracic scoliosis. The location of apical vertebra and the severity of scoliosis are the risk factors influencing the pulmonary dysfunction.

关 键 词：脊柱侧凸 神经纤维瘤病1型 呼吸功能试验 

分 类 号：R739.4[医药卫生—肿瘤] R682[医药卫生—临床医学]