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杜氏肌营养不良症中的睡眠呼吸障碍。

Sleep disordered breathing in Duchenne muscular dystrophy.

机构信息

Department of Pediatrics, University of Cincinnati, College of Medicine, Cincinnati, OH, United States; Division of Pulmonology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, United States.

出版信息

Paediatr Respir Rev. 2019 Apr;30:2-8. doi: 10.1016/j.prrv.2018.07.003. Epub 2018 Jul 18.

Abstract

Symptoms of sleep disordered breathing (SDB) in younger boys with DMD are often poorly perceived and/or articulated by the patients or their families. As a result it is the watchful eye of the care-provider that determines the need for early polysomnographic (PSG) assessments. The use of polysomnography without capnometry should be considered completely inadequate when it comes to diagnosis and management of SDB in these patients. The stabilization of gas exchange with non-invasive ventilation may be achieved by the use of pressure or volume support ventilation. Serial PSG assessments are recommended to assure optimal management as the patients' clinical status evolves with disease progression and the emergence of additional morbidities such as cardiomyopathies, dysphagia, and chronic aspiration.

摘要

患有 DMD 的年轻男孩的睡眠呼吸紊乱 (SDB) 症状通常难以被患者或其家属察觉和/或表达。因此,需要护理提供者密切关注,以确定是否需要早期进行多导睡眠图 (PSG) 评估。对于这些患者的 SDB 的诊断和管理,不使用二氧化碳描记法的多导睡眠图的使用应被认为是完全不充分的。通过使用压力或容量支持通气,可以实现气体交换的稳定。建议进行连续 PSG 评估,以确保在患者的临床状况随着疾病进展和出现其他并发症(如心肌病、吞咽困难和慢性吸入)而演变时,进行最佳管理。

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