1 Department of Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Chron Respir Dis. 2017 Nov;14(4):392-406. doi: 10.1177/1479972317694621. Epub 2017 Mar 6.
Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder associated with severely impaired mucociliary clearance caused by defects in ciliary structure and function. Although recurrent bacterial infection of the respiratory tract is one of the major clinical features of this disease, PCD airway microbiology is understudied. Despite the differences in pathophysiology, assumptions about respiratory tract infections in patients with PCD are often extrapolated from cystic fibrosis (CF) airway microbiology. This review aims to summarize the current understanding of bacterial infections in patients with PCD, including infections with Pseudomonas aeruginosa, Staphylococcus aureus, and Moraxella catarrhalis, as it relates to bacterial infections in patients with CF. Further, we will discuss current and potential future treatment strategies aimed at improving the care of patients with PCD suffering from recurring bacterial infections.
原发性纤毛运动障碍(PCD)是一种常染色体隐性遗传病,与纤毛结构和功能缺陷导致的严重黏液纤毛清除功能障碍有关。虽然呼吸道反复细菌感染是该病的主要临床特征之一,但 PCD 气道微生物组学的研究却很少。尽管病理生理学存在差异,但人们对 PCD 患者呼吸道感染的假设通常是从囊性纤维化(CF)气道微生物组学中推断出来的。本综述旨在总结目前对 PCD 患者细菌感染的认识,包括铜绿假单胞菌、金黄色葡萄球菌和卡他莫拉菌感染,以及其与 CF 患者细菌感染的关系。此外,我们还将讨论当前和潜在的未来治疗策略,旨在改善反复发生细菌感染的 PCD 患者的治疗。