Atypical basal bodies Found in ciliated cells of an asthmatic patient: An acquired ultrastructural alteration or evidence of a disorder of ciliogenesis?

Author(s):  
W.T. Gunning ◽  
R.F. Judkins ◽  
B.E. Akpunonu

Numerous reports of abnormal cilia in human respiratory epithelium have been published within the last ten years; most of these studies have described alterations of the axoneme and attributed these changes to a dysfunction of the ciliary apparatus, otherwise labeled immotile cilia syndrome or primary ciliary dyskinesia. In general, dysfunctional or ineffective mucociliary clearing of the respiratory tract is a prime factor contributing to chronic respiratory tract infections, and while patients with chronic respiratory tract infections do not necessarily have an immotile cilia syndrome, this history is the norm for patients suffering from the congenital disorder. More than 20 alterations of the cilia axoneme have been observed. The most common is the loss of dynein arms and other frequently seen abnormalities include loss of radial spokes and transposition of ciliary microtubules. Approximately one half of the patients found to have ciliary abnormalities also have situs inversus, bronchiectasis, and chronic sinusitis, a triad of manifestations known as Kartagener's syndrome.

2017 ◽  
Vol 14 (4) ◽  
pp. 392-406 ◽  
Author(s):  
Christiaan DM Wijers ◽  
James F Chmiel ◽  
Benjamin M Gaston

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.


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