关键词: Primary ciliary dyskinesia Pulmonary management Pulmonary manifestations Respiratory complications

Mesh : Humans Kartagener Syndrome / diagnosis therapy physiopathology complications Bronchiectasis / therapy physiopathology diagnosis Sinusitis / therapy diagnosis physiopathology Otitis Media with Effusion / diagnosis physiopathology therapy Pneumonia / diagnosis therapy physiopathology

来  源:   DOI:10.1016/j.ccm.2024.02.020

Abstract:
This review article explores the respiratory aspects of primary ciliary dyskinesia (PCD), a rare, heterogenous, genetic disorder characterized by impaired motile ciliary function. It discusses the clinical diagnosis and management strategies for PCD-related respiratory disease, including chronic sinusitis, otitis media with effusion, recurrent pneumonia, and bronchiectasis. The review emphasizes the need for a multidisciplinary approach to optimize care and clinical trials to improve outcomes in individuals with PCD, highlighting the importance of accurate diagnosis.
摘要:
这篇综述文章探讨了原发性纤毛运动障碍(PCD)的呼吸方面,一种罕见的,异质,以活动纤毛功能受损为特征的遗传性疾病。讨论了PCD相关呼吸系统疾病的临床诊断和管理策略,包括慢性鼻窦炎,渗出性中耳炎,复发性肺炎,还有支气管扩张.该综述强调需要采用多学科方法来优化护理和临床试验,以改善PCD患者的预后。强调准确诊断的重要性。
公众号