AI Article Synopsis
- Primary ciliary dyskinesia (PCD) is a genetic disorder that causes repeated airway infections, and there are currently no specific treatments available for it.
- This study analyzed neutrophils (a type of immune cell) from both the sputum of PCD patients and their blood, finding that sputum neutrophils were highly activated but were unresponsive to normal stimuli.
- The research suggests that dysfunctional neutrophils in PCD may worsen inflammation in the airways, and further studies should explore anti-inflammatory treatments and ways to enhance the clearance of dead cells.
Article Abstract
Background: Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by recurrent airway infection and inflammation. There is no cure for PCD and to date there are no specific treatments available. Neutrophils are a crucial part of the immune system and are known to be dysfunctional in many inflammatory diseases. So far, the role of the neutrophils in PCD airways is largely unknown. The purpose of this study was to investigate the phenotype and function of airway neutrophils in PCD, and compare them to blood neutrophils.
Methods: Paired peripheral blood and spontaneously expectorated sputum samples from patients with PCD (n = 32) and a control group of patients with non-PCD, non-cystic fibrosis bronchiectasis (n = 5) were collected. The expression of neutrophil-specific surface receptors was determined by flow cytometry. Neutrophil function was assessed by measuring the extent of actin polymerization, production of reactive oxygen species (ROS) and release of neutrophil extracellular traps (NETs) in response to activating stimuli.
Results: Sputum neutrophils displayed a highly activated phenotype and were unresponsive to stimuli that would normally induce ROS production, actin polymerization and the expulsion of NETs. In addition, PCD sputum displayed high activity of neutrophil elastase, and impaired the efferocytosis by healthy donor macrophages.
Conclusions: Sputum neutrophils in PCD are dysfunctional and likely contribute to ongoing inflammation in PCD airways. Further research should focus on anti-inflammatory therapies and stimulation of efferocytosis as a strategy to treat PCD.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9758951 | PMC |
| http://dx.doi.org/10.1186/s12931-022-02280-7 | DOI Listing |
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