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[原发性纤毛运动障碍的物理治疗]

[Physiotherapy in Primary Ciliary Dyskinesia].

作者信息

Krämer Christina, Flock Jasmin, Borges-Lüke Birgit, Raidt Johanna, Ringshausen Felix C

机构信息

Zentrale Einrichtung Therapeutische Gesundheitsberufe, Universitätsklinikum Münster, Münster, Deutschland.

Praxis für Klein & Groß, Physiotherapie Kirbach, Duisburg, Deutschland.

出版信息

Pneumologie. 2025 Aug;79(8):577-584. doi: 10.1055/a-2502-8151. Epub 2025 Apr 1.

Abstract

Primary ciliary dyskinesia (PCD) is a multisystem disease caused by dysfunction of motile cilia. It is characterized by chronic mucus retention of the upper and lower airways. This results in a destructive lung disease with the development of bronchiectasis. There are a very few evidence-based therapies for patients with PCD. Most treatment concepts are based on other respiratory diseases or expert opinion. This article is about the physiotherapy care of patients with PCD and provides an overview of recommendations from the current literature as well as many years of experience in the physiotherapy treatment of patients with PCD. Currently, no curative therapy for PCD exists. Symptomatic treatment with a focus on mucus management is relevant. This includes inhalation, mobilization of mucus, physical activity and the targeted use of medical aids for chest clearance as well as rehabilitation. Whenever possible, specialized therapists should provide guidance for physiotherapy.

摘要

原发性纤毛运动障碍(PCD)是一种由运动性纤毛功能障碍引起的多系统疾病。其特征为上、下呼吸道慢性黏液潴留。这会导致一种具有支气管扩张发展的破坏性肺部疾病。对于PCD患者,基于证据的治疗方法非常少。大多数治疗理念基于其他呼吸系统疾病或专家意见。本文探讨PCD患者的物理治疗护理,并概述当前文献中的建议以及多年来PCD患者物理治疗的经验。目前,尚无治愈PCD的疗法。以黏液管理为重点的对症治疗至关重要。这包括吸入、黏液清除、体育活动、有针对性地使用胸部排痰医疗辅助工具以及康复治疗。只要有可能,专业治疗师应为物理治疗提供指导。

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