肌营养不良患儿的多导睡眠监测的特征

Characteristics of polysomnography in children with muscular dystrophy

目的分析肌营养不良(MD)患儿夜间多导睡眠监测(PSG)的表现及特征,为适时的呼吸支持提供依据。方法回顾性分析2017年1月至2018年12月在北京大学第一医院神经肌肉病多学科联合门诊就诊,经临床、肌电图、基因或病理确诊的MD且进行了整夜PSG监测患儿的资料。分析其PSG监测特征。结果总计入组10例MD患儿,其中男7例,女3例,行PSG监测时年龄(9.40±3.13)岁,均经临床、病理和基因检测确诊为MD。MD患儿在快速眼球运动(REM)睡眠期的呼吸暂停低通气指数(AHI)较非快速眼球运动(NREM)睡眠期呈增高趋势。在呼吸事件方面,MD患儿可表现阻塞性呼吸暂停、中枢型呼吸暂停、混合型呼吸暂停和低通气事件,其中低通气指数(HI)最高,混合型呼吸暂停指数(MSAI)最低,HI显著高于MSAI(P<0.05)。REM睡眠期的中枢型呼吸暂停不排除膈肌性呼吸事件的可能。在睡眠结构方面,PSG显示MD患儿REM睡眠时间减少。结论MD患儿的早期睡眠呼吸紊乱,表现为以低通气事件为主,呼吸紊乱最早出现于REM睡眠期,REM期的呼吸紊乱指数可作为MD患儿呼吸肌受累的早期参考指标。

Objective To analyze the manifestations and characteristics of nocturnal polysomnography(PSG)in children with muscular dystrophy(MD),and to provide evidence for timely respiratory support.Methods The clinical data and PSG study characteristics were retrospectively analyzed of the children with MD diagnosed by clinical,electromyographic,genetic,or pathological confirmation from January 2017 to December 2018 at the multidisciplinary outpatient clinic of neuromuscular disease at Peking University First Hospital.Results A total of 10 children with MD were recruited,including 7 males and 3 females,aged(9.40±3.13)years at the time of PSG study,who were diagnosed with MD by clinical,pathological and genetic testing.Children with MD tend to have a higher apnea-hypopnea index(AHI)during the rapid eye movement(REM)sleep compared to the non-rapid eye movements(NREM)sleep.In terms of respiratory events,children with MD can exhibit obstructive apnea,central apnea,mixed apnea,and hypopnea,with the highest hypopnea index(HI)and the lowest mixed apnea index(MSAI),with HI significantly hi-gher than MSAI(P<0.05).Partial central apnea during REM sleep may be the diaphragmatic"pseudo"central respiratory events.In terms of sleep architecture,PSG showed reduced REM sleep duration in children with MD.Conclusions The main respiratory disorder in children with MD is hypopnea.Respiratory disorder occur firstly during REM sleep.Respiratory disorder index in REM sleep may be used as an early warning indicator for the involvement of respiratory muscles in children with MD.