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与先天性罕见疾病梅比厄斯综合征和波兰综合征特征相关的睡眠呼吸障碍。

Sleep-related breathing disorders associated with the characteristics of underlying congenital rare diseases of Moebius syndrome and Poland syndrome.

作者信息

Sugiyama Ai Inagaki, Shiota Satomi, Yamada Tomoko, Ito Jun, Suzuki Kazuhiro, Takahashi Kazuhisa

机构信息

Division of Respiratory Medicine Juntendo University Faculty of Medicine & Graduate School of Medicine Tokyo Japan.

Division of Radiology Juntendo University Faculty of Medicine & Graduate School of Medicine Tokyo Japan.

出版信息

Respirol Case Rep. 2020 May 18;8(5):e00579. doi: 10.1002/rcr2.579. eCollection 2020 Jul.

Abstract

A 24-year-old woman was referred to us with daytime sleepiness. She has two congenital intractable and rare diseases, namely, Moebius syndrome and Poland syndrome. Physiological examinations and a detailed usage analysis under a ventilation device helped to conclude that hypoglossal nerve paralysis and thoracic deformity from her two underlying diseases were associated closely with her final diagnosis of obstructive sleep apnoea and sleep-related hypoventilation due to medical disorders. Bilevel positive pressure ventilation with auto-titrating expiratory positive airway pressure was effective. This is the first report that describes in detail the causal interactions between underlying two intractable and rare diseases and sleep-related breathing disorders.

摘要

一名24岁女性因日间嗜睡前来就诊。她患有两种先天性难治性罕见疾病,即默比厄斯综合征和波兰综合征。生理检查及通气设备详细使用分析表明,其两种基础疾病所致的舌下神经麻痹和胸廓畸形与最终诊断的因疾病引起的阻塞性睡眠呼吸暂停和睡眠相关通气不足密切相关。双水平正压通气结合自动调压呼气末正压通气有效。这是首份详细描述两种基础难治性罕见疾病与睡眠相关呼吸障碍之间因果关系的报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/748e/7235444/e404e809c753/RCR2-8-e00579-g001.jpg

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