Bacterial infections in patients with primary ciliary dyskinesia: Comparison with cystic fibrosis

Chron Respir Dis. 2017 Nov;14(4):392-406. doi: 10.1177/1479972317694621. Epub 2017 Mar 6.

Abstract

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.

Keywords: CF; PCD; airways; infections; microbiology.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Achromobacter denitrificans / isolation & purification
  • Bacteria, Anaerobic / isolation & purification
  • Bacterial Infections / drug therapy
  • Bacterial Infections / microbiology*
  • Cystic Fibrosis / complications*
  • Haemophilus influenzae / isolation & purification
  • Humans
  • Kartagener Syndrome / complications*
  • Moraxella catarrhalis / isolation & purification
  • Nontuberculous Mycobacteria / isolation & purification
  • Pseudomonas aeruginosa / isolation & purification
  • Ralstonia / isolation & purification
  • Respiratory System / microbiology*
  • Respiratory Tract Infections / microbiology*
  • Staphylococcus aureus / isolation & purification
  • Streptococcus pneumoniae / isolation & purification