Department of Rehabilitation and Sports Medicine, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
Neuromuscul Disord. 2012 Dec;22(12):1083-9. doi: 10.1016/j.nmd.2012.05.008. Epub 2012 Jun 23.
We assessed pulmonary function in hereditary motor and sensory neuropathy. Fourteen neuropathy patients without spinal deformity (group 1), 14 with spinal deformity (group 2), and 16 individuals with idiopathic spinal deformity (group 3) matched to group 2 for age, height and Cobb angle, were included. Hereditary motor and sensory neuropathy severity was measured with Charcot-Marie-Tooth Neuropathy Score. All participants exhibited mild decrease in maximal inspiratory pressure at the mouth. One-way analysis of variance yielded significant main effects for lung volumes - slow vital capacity, forced expiratory volume in 1s, and total lung capacity (p's<.01), attributable to greater volumes in group 1 compared to groups with spinal deformity - and transfer factor for carbon monoxide (p=.013), reflecting differences between groups 1 vs. 2. Slow vital capacity and total lung capacity correlated with maximal inspiratory pressure at the mouth in group 2, whereas slow vital capacity correlated with muscle work in group 3 (p's<.05). Decreased lung volume may be due to impaired respiratory muscle strength in hereditary motor and sensory neuropathy with spinal deformity and due to spinal deformity in idiopathic patients.
我们评估遗传性运动感觉神经病的肺功能。14 名无脊柱畸形的神经病患者（第 1 组）、14 名有脊柱畸形的神经病患者（第 2 组）和 16 名与第 2 组年龄、身高和 Cobb 角匹配的特发性脊柱畸形患者（第 3 组）纳入研究。遗传性运动感觉神经病的严重程度采用 Charcot-Marie-Tooth 神经病评分进行测量。所有参与者在口部最大吸气压力均表现出轻度下降。单因素方差分析得出肺活量-缓慢肺活量、1 秒用力呼气量和总肺活量（p<.01）的主要效应显著，这归因于第 1 组的容量大于脊柱畸形组，一氧化碳转移因子（p=.013）反映了第 1 组与第 2 组之间的差异。在第 2 组中，缓慢肺活量和总肺活量与口部最大吸气压力相关，而在第 3 组中，缓慢肺活量与肌肉工作相关（p<.05）。在有脊柱畸形的遗传性运动感觉神经病中，肺容积减少可能是由于呼吸肌力量受损所致，而在特发性患者中则是由于脊柱畸形所致。
