儿童期发作性睡病合并 Prader-Willi 综合征患儿的睡眠呼吸障碍。
Sleep-disordered breathing in school-aged children with Prader-Willi syndrome.
机构信息
Melbourne Children's Sleep Centre, Monash Children's Hospital, Monash Health, Melbourne, Australia.
Department of Pediatrics, Monash University, Melbourne, Australia.
出版信息
J Clin Sleep Med. 2022 Apr 1;18(4):1055-1061. doi: 10.5664/jcsm.9788.
STUDY OBJECTIVES
Studies of sleep-disordered breathing (SDB) in children with Prader-Willi syndrome (PWS) have focused on early childhood and growth hormone (GH)-naïve children, but little is known about older children, including those on long-term GH therapy. This study aimed to describe the nature and prevalence of SDB in school-aged children with PWS in the growth hormone era.
METHODS
This retrospective single-center chart review included children aged 6-18 years with PWS who had overnight polysomnography not involving respiratory support over 5 years (2012-2017). The main outcome measures were the presence of obstructive sleep apnea, central sleep apnea, or hypoventilation defined by an elevated transcutaneous partial pressure of carbon dioxide (TcPCO) as per standard pediatric criteria.
RESULTS
Seventeen children (8 males; median age 11.6 years, range 6.6-16.1 years) were included. Fifteen demonstrated SDB of different types: central sleep apnea (18%), obstructive sleep apnea (24%), both obstructive and central sleep apnea (29%), or hypoventilation without obstructive or central sleep apnea (18%). Twelve (71%) children had evidence of hypoventilation. Those with hypoventilation had a higher central apnea-hypopnea index but no difference in the obstructive apnea-hypopnea index, age, sex, growth parameters, or the presence of scoliosis or sleep-related symptoms compared with those without hypoventilation.
CONCLUSIONS
Sleep-related hypoventilation is common in school-aged children with PWS. The presence of central sleep apnea, including the quantification of central hypopneas, but not obstructive sleep apnea or clinical factors, predicted the presence of hypoventilation. Long-term polysomnography surveillance in children with PWS should include identification of central hypopneas and measurement of continuous pCO.
CITATION
Schaefer J, Davey MJ, Nixon GM. Sleep-disordered breathing in school-aged children with Prader-Willi syndrome. . 2022;18(4):1055-1061.
研究目的
患有普拉德-威利综合征（PWS）的儿童的睡眠呼吸障碍（SDB）研究集中在幼儿和生长激素（GH）初治儿童，但对于年龄较大的儿童，包括长期接受 GH 治疗的儿童，知之甚少。本研究旨在描述 GH 时代学龄期 PWS 儿童 SDB 的性质和流行情况。
方法
本回顾性单中心图表回顾包括在过去 5 年中（2012-2017 年）接受过不涉及呼吸支持的过夜多导睡眠图检查的 6-18 岁 PWS 儿童。主要结局指标为阻塞性睡眠呼吸暂停、中枢性睡眠呼吸暂停或高碳酸血症（TcPCO）升高定义的通气不足，符合标准儿科标准。
结果
共纳入 17 名儿童（8 名男性；中位年龄 11.6 岁，范围 6.6-16.1 岁）。15 名儿童表现出不同类型的 SDB：中枢性睡眠呼吸暂停（18%）、阻塞性睡眠呼吸暂停（24%）、阻塞性和中枢性睡眠呼吸暂停（29%）或无阻塞性或中枢性睡眠呼吸暂停的通气不足（18%）。12 名（71%）儿童存在通气不足的证据。与无通气不足的儿童相比，通气不足的儿童的中枢性呼吸暂停低通气指数较高，但阻塞性呼吸暂停低通气指数、年龄、性别、生长参数、脊柱侧凸或睡眠相关症状无差异。
结论
睡眠相关通气不足在学龄期 PWS 儿童中很常见。中枢性睡眠呼吸暂停的存在，包括对中枢性低通气的定量，但不是阻塞性睡眠呼吸暂停或临床因素，预测了通气不足的存在。PWS 儿童的长期多导睡眠图监测应包括识别中枢性低通气和测量连续 pCO。
引文
Schaefer J、Davey MJ、Nixon GM。患有普拉德-威利综合征的学龄儿童的睡眠呼吸障碍。J Clin Sleep Med. 2022;18(4):1055-1061.
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