摘要
目的探讨脊髓性肌萎缩(spinal muscular atrophy,SMA)患儿肺功能改变的特征。方法选取参加2016年首都儿科研究所附属儿童医院与北京市美儿SMA关爱中心举办的"关爱SMA患儿"的公益活动的16例SMA患儿作为研究组,应用肺量计进行用力呼气流量-容积曲线测定,参数包括用力呼气肺活量(forced vital capacity,FVC)、第1秒用力呼气容积(forced expiratory volumeatonesecond,FEV_1)、1秒率(FEV_1/FVC)、用力呼气峰流量(peak expiratory flow,PEF)、用力呼气中期流量(maximal mid-expiratory?ow rate,MMEF)等。测量结果除FEV_1/FVC为实测值外,其余均以实测值占预计值的百分比表示。将研究组患儿按临床病情严重度分为轻度组(10例)和重度组(6例),进一步分析其肺功能损害情况。选取同期幼儿园和在校的16例健康儿童作为对照组,比较分析两组儿童的肺功能情况。结果研究组中4例患儿肺通气功能正常,12例患儿存在不同程度的肺功能损害,其中9例患儿FVC明显降低,仅3例患儿FVC在正常范围。与对照组比较,SMA患儿FVC、FEV_1、PEF、MMEF均显著降低(P<0.01),FEV_1/FVC在正常范围,虽低于对照组,但差异无显著性(P=0.070)。通气功能正常的4例SMA患儿的PEF[(90.2±6.0)%]亦低于对照组[(113.6±13.4)%,P=0.003]。SMA患儿中,通气异常与通气功能正常的患儿比较,仅FEV_1/FVC未见显著差异(P=0.308),其他各指标均明显降低(P<0.01)。重度组SMA患儿的FVC、FEV_1明显低于轻度组(P<0.05)。结论多数SMA患儿肺功能存在不同程度降低,其中反映呼吸肌力的敏感指标PEF首先出现降低。肺功能呈明确的以肺容积FVC、FEV_1同时下降为特征的限制型通气功能障碍。因此,监测应以FVC、PEF、FEV_1等反映通气、呼吸肌力的参数为重点,从而为病情评估和临床管理提供参照。
Objective To investigate the characteristics of lung function changes in children with spinal muscular atrophy(SMA).Method 16 children with SMA were mainly from the event they participated in 2016 organized by the Children’s Hospital of Capital Institute of Pediatrics and the Spinal Muscular Atrophy Care Center.The spirometer was used to measure the forced expiratory flow-volume curve.The parameters included forced expiratory vital capacity(FVC),forced expiratory volume in 1 second(FEV1),1 second rate(FEV1/FVC),and peak expiratory flow(PEF),maximal mid-expiratory flow(MMEF).The measured results were expressed as a percentage of the measured values except for the 1 second rate.16 cases of healthy children in kindergarten and school were selected for comparison to observe the changes of lung function in children with SMA.Children with SMA were divided into mild(10 cases)and severe(6 cases)groups according to the severity of clinical disease,and their lung function damage was further analyzed.Result Among the 16 children with SMA enrolled,4 had normal pulmonary ventilation function,12 had different degrees of pulmonary dysfunction,and 9 had significantly lower FVC.Only 3 patients had normal FVC.Compared with healthy controls,FVC,FEV1,PEF,and MMEF were significantly lower in children with SMA(P<0.01),and their FEV1/FVC was in the normal range,but still lower than the healthy control group(P=0.070).The PEF[(90.2±6.0)%]of 4 children with SMA with normal ventilation function was also lower than that of the healthy control group[(113.6±13.4)%,P=0.003].Among children with SMA,there was no significant difference in FEV1/FVC between the ventilatory dysfunction and normal groups(P=0.308),the other parameters were significantly lower(P<0.01).The parameters of lung function in children with severe SMA were significantly lower than that in the mild group(P<0.05).Conclusion Most of the children with SMA have different degrees of decreased lung function,and the PEF which sensitive to respiratory muscle strength reduced first,
作者
李硕
郭文卉
刘传合
姜欣玫
曹玲
LI Shuo;GUO Wen-hui;LIU Chuan-he;JIANG Xin-mei;CAO Ling(Department of Allergy,Capital Institute of Pediatrics,Beijing 100020,China;Department of Respiration,Capital Institute of Pediatrics,Beijing 100020,China)
出处
《中国医刊》
CAS
2019年第5期536-540,共5页
Chinese Journal of Medicine
基金
北京市医院管理局重点医学专业发展计划(ZYLX201829)
关键词
脊髓性肌萎缩
肺功能
限制型通气功能障碍
Spinal muscular atrophy
Pulmonary function
Restricted ventilatory dysfunction
