无创双水平正压通气治疗先天性中枢性低通气综合征1例并文献复习

发育医学电子杂志

2013, Vol. 1

Issue (2): 87-90

结构畸形论著

无创双水平正压通气治疗先天性中枢性低通气综合征1例并文献复习

李秋平刘敬何玺玉杨尧韩涛梁婧封志纯

京军区总医院附属八一儿童医院NICIf，
北京100700)

Non-invasive bi-level airway pressureventilation for central hypoVentnation syndrome：a csse report and fiterature review

L1#br# Qiu-ping,LIU Jing,HE Xi-yu，YANG Yao#br#

NICU Bayi
Children's Hospital Affiliated to the General Hospital of Chinese PLA Beijing Military Region．Beijing
100700,China

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摘要【摘要】目的应用无创双水平正压通气(BiPAP)治疗先天性中枢性低通气综合征(CCHS)1例
的成功经验，并复习文献，以提高对该病的认识和临床诊疗水平。方法对l例确诊为CCHS的患儿
的临床资料和无创通气治疗情况进行总结，并结合文献资料进行分析。结果患儿生后当灭以呼吸
困难起病，呈现明显睡眠时通气障碍，反复撤机困难，基因检测证实存在Phox2b基因突变，确诊为
CCHS。予以无创BiPAP治疗，经皮血氧饱和度及TCO：维持良好，患儿于3个月顺利出院，继续家
庭无创通气。随访至今已4个月，生长发育良好。结论无创BiPAP可为CCHS患儿提供有效的呼吸
支持，可避免既往气管切开带来的诸多不良后果。

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关键词: 无创通气；先天性中枢性低通气综合征；婴儿新生

Abstract: 【Abstract】Objectives
To
report
an infant with central hypoventilation
syndrome
treated by non—invasive
bi—level airway pressure ventilation and review the related 1iterature．Methods Clinieal
data of an infant with
CCHS treated bv BiPAP and the related literature were collected and analyzed．Results A female infant showed
a significant
dyspnea
after birth and
obviously
ventilator impairment in sleep，with repeated weaning failure．A
PHOX2B mutation was indentified in this baby and confirmed
as CCHS．She was
supported by
non-invasive
BiPAP with normal pulse oximetry(Sp02)and transcutaneous partial pressure of carbon dioxide(TepC02)．
The infant was
discharged
home at 3 months of
age
with non—invasive BiPAP and showed normal development
after 4 months of follow up．Conclusion Non-invasive BiPAP is one of the effective methods in the treatment of
CCHS and can avoid the adverse consequences caused by tracheotomy．

Key words: n—invasive ventilation Congenital
central hypoventilation syndrome Newborn

出版日期: 2013-04-30 发布日期: 2019-09-07 期的出版日期: 2013-04-30

通讯作者: 封志纯 https://baike.baidu.com/item/%E5%B0%81%E5%BF%97%E7%BA%AF/9328206?fr=aladdin E-mail: Email：zhjfengzc@126．com

引用本文:

李秋平刘敬何玺玉杨尧韩涛梁婧封志纯. 无创双水平正压通气治疗先天性中枢性低通气综合征1例并文献复习[J]. 发育医学电子杂志, 2013, 1(2): 87-90.
L Qiu-ping, LIU Jing, HE Xi-yu, YANG Yao. Non-invasive bi-level airway pressureventilation for central hypoVentnation syndrome：a csse report and fiterature review. Journal of Developmental Medicine(Electronic Version), 2013, 1(2): 87-90.

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http://www.fyyxzz.com/CN/ 或 http://www.fyyxzz.com/CN/Y2013/V1/I2/87

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