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本文引用的文献

1
Primary Ciliary Dyskinesia in Adult Bronchiectasis: Data from the German Bronchiectasis Registry PROGNOSIS.成人支气管扩张症中的原发性纤毛运动障碍:来自德国支气管扩张症注册研究 PROGNOSIS 的数据。
Chest. 2024 Nov;166(5):938-950. doi: 10.1016/j.chest.2024.05.023. Epub 2024 Jun 15.
2
Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype-phenotype correlations.对1236名基因分型的原发性纤毛运动障碍患者的分析确定了不同DNA变异的区域聚类以及显著的基因型-表型相关性。
Eur Respir J. 2024 Aug 8;64(2). doi: 10.1183/13993003.01769-2023. Print 2024 Aug.
3
[Primary ciliary dyskinesia].[原发性纤毛运动障碍]
Inn Med (Heidelb). 2024 Jun;65(6):545-559. doi: 10.1007/s00108-024-01726-y. Epub 2024 May 27.
4
Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).支气管扩张症患者的气道清除管理:来自欧洲支气管扩张症注册研究(EMBARC)的数据。
Eur Respir J. 2024 Jun 6;63(6). doi: 10.1183/13993003.01689-2023. Print 2024 Jun.
5
Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial.上皮钠通道阻滞剂依德氯铵在原发性纤毛运动障碍患者中的安全性和有效性(CLEAN-PCD):一项多国、2期、随机、双盲、安慰剂对照交叉试验。
Lancet Respir Med. 2024 Jan;12(1):21-33. doi: 10.1016/S2213-2600(23)00226-6. Epub 2023 Aug 31.
6
Cystic fibrosis and primary ciliary dyskinesia: Similarities and differences.囊性纤维化和原发性纤毛运动障碍:相似与不同。
Respir Med. 2023 Apr;209:107169. doi: 10.1016/j.rmed.2023.107169. Epub 2023 Feb 22.
7
Primary ciliary dyskinesia: A multicenter survey on clinical practice and patient management in Italy.原发性纤毛运动障碍:意大利临床实践与患者管理的多中心调查
Pediatr Pulmonol. 2023 Apr;58(4):1127-1135. doi: 10.1002/ppul.26303. Epub 2023 Jan 9.
8
Physical activity, respiratory physiotherapy practices, and nutrition among people with primary ciliary dyskinesia in Switzerland - a cross-sectional survey.瑞士原发性纤毛运动障碍患者的体力活动、呼吸物理治疗实践和营养状况-一项横断面调查。
Swiss Med Wkly. 2022 Aug 18;152:w30221. doi: 10.4414/smw.2022.w30221. eCollection 2022 Aug 15.
9
The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN.原发性纤毛运动障碍疾病特异性临床试验网络:PCD-CTN
ERJ Open Res. 2022 Aug 15;8(3). doi: 10.1183/23120541.00139-2022. eCollection 2022 Jul.
10
Respiratory symptoms of Swiss people with primary ciliary dyskinesia.瑞士原发性纤毛运动障碍患者的呼吸道症状
ERJ Open Res. 2022 Apr 11;8(2). doi: 10.1183/23120541.00673-2021. eCollection 2022 Apr.

[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.

DOI:10.1055/a-2502-8151
PMID:40169130
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12356653/
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。