The Antimicrobial Activity of Peripheral Blood Neutrophils Is Altered in Patients with Primary Ciliary Dyskinesia

The airways of patients with primary ciliary dyskinesia (PCD) contain persistently elevated neutrophil numbers and CXCL8 levels. Despite their abundance, neutrophils fail to clear the airways from bacterial infections. We investigated whether neutrophil functions are altered in patients with PCD. Ne...

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Bibliographic Details
Published in:International journal of molecular sciences 2021-06, Vol.22 (12), p.6172
Main Authors: Cockx, Maaike, Blanter, Marfa, Gouwy, Mieke, Ruytinx, Pieter, Abouelasrar Salama, Sara, Knoops, Sofie, Pörtner, Noëmie, Vanbrabant, Lotte, Lorent, Natalie, Boon, Mieke, Struyf, Sofie
Format: Article
Language:English
Subjects:
antibacterial activity
Antiinfectives and antibacterials
Antimicrobial activity
Antimicrobial agents
Bacteria
Blood
Chemokines
Chemotaxis
Chronic obstructive pulmonary disease
Cystic fibrosis
Cytokines
Dyskinesia
Inflammation
Leukocytes (neutrophilic)
Lungs
Neutrophils
Pathogenesis
Pathogens
Pediatrics
Peripheral blood
Phagocytes
Primary ciliary dyskinesia
Reactive oxygen species
Receptor mechanisms
Respiratory diseases
Stimulation
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Summary:The airways of patients with primary ciliary dyskinesia (PCD) contain persistently elevated neutrophil numbers and CXCL8 levels. Despite their abundance, neutrophils fail to clear the airways from bacterial infections. We investigated whether neutrophil functions are altered in patients with PCD. Neutrophils from patients and healthy controls (HC) were isolated from peripheral blood and exposed to various bacterial stimuli or cytokines. Neutrophils from patients with PCD were less responsive to low levels of fMLF in three different chemotaxis assays (p < 0.05), but expression of the fMLF receptors was unaltered. PCD neutrophils showed normal phagocytic function and expression of adhesion molecules. However, PCD neutrophils produced less reactive oxygen species upon stimulation with bacterial products or cytokines compared to HC neutrophils (p < 0.05). Finally, the capacity to release DNA, as observed during neutrophil extracellular trap formation, seemed to be reduced in patients with PCD compared to HC (p = 0.066). These results suggest that peripheral blood neutrophils from patients with PCD, in contrast to those of patients with cystic fibrosis or COPD, do not show features of over-activation, neither on baseline nor after stimulation. If these findings extend to lung-resident neutrophils, the reduced neutrophil activity could possibly contribute to the recurrent respiratory infections in patients with PCD.
ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms22126172