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儿童先天性中枢性低通气综合征和其他原因引起的睡眠相关低通气

Author
Robert T Brouillette, MD
Section Editor
Ronald D Chervin, MD, MS
Deputy Editor
Alison G Hoppin, MD
Translators
常丽, 副主任医师

引言

低通气(通气不足)可由脑、脊髓、神经、肌肉、心脏、肺或气道的疾病引起。睡眠相关低通气的临床表现为主要发生在睡眠过程中的通气不足。受累个体存在低通气引起低氧血症和心动过缓的风险，需要在睡眠过程中持续监测是否出现这些问题。

儿童睡眠过程中低通气最常见的原因是阻塞性睡眠呼吸暂停(obstructive sleep apnea, OSA)，相关内容将在其他专题中讨论。 (参见“儿童疑似阻塞性睡眠呼吸暂停的评估”和“儿童阻塞性睡眠呼吸暂停的治疗”)

非阻塞性睡眠相关低通气要少见得多，其通常是由几种罕见的遗传性或神经性通气调节异常之一引起的，特别是先天性中枢性低通气综合征(congenital central hypoventilation syndrome, CCHS)，迟发性中枢性低通气综合征(late onset central hypoventilation syndrome, LO-CHS)，或者快速肥胖伴下丘脑功能障碍、通气不足和自主神经失调(rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation, ROHHAD)综合征。本专题将讨论这些疾病。

定义

●低通气是指通气器官清除二氧化碳(CO2)与代谢产生CO2之间出现失衡。一般来说，低通气定义为清醒患者的动脉血CO2分压(pCO2)高于正常值(35-45mmHg)。低通气常常会伴有低氧血症，但不总是如此。基于以下情况，临床医生可能会怀疑低通气：毛细血管或静脉血气分析显示原因不明的CO2分压升高(>50mmHg)和碳酸氢盐水平升高(>25mEq/L)，或者静息状态下脉搏血氧测定显示基线血氧饱和度小于96%。

●睡眠相关低通气是指在睡眠过程中加重或仅发生在睡眠过程中的低通气。低通气往往发生在白天小睡和夜间睡眠中，因此术语“睡眠相关低通气”优于“夜间低通气”。睡眠过程中，因为没有觉醒呼吸驱动，健康个体的动脉血pCO2(PaCO2)会上升至高达50mmHg。

                          

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Literature review current through: 2017-06 . | This topic last updated: 2017-05-16.
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