Dysfunctions of Neutrophils in the Peripheral Blood of Children with Cystic Fibrosis

Bakalović, Ganimeta; Bokonjić, Dejan; Mihajlović, Dušan; Čolić, Miodrag; Mališ, Vanja; Drakul, Marija; Tomić, Sergej; Jojić, Ivan; Rakočević, Sara; Popović, Darinka; Kozić, Ljiljana; Vasiljević, Miloš; Bekić, Marina; Mašić, Srđan; Ljuboja, Olivera

doi:10.3390/biomedicines11061725

Cited by 1 publication

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References 88 publications

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“…Bakalović et al studied the functional and phenotypic changes in neutrophils in children with CF ( 16 ). This study included 19 CF children (ages 5–17 years) and 14 age-matched healthy children.…”

Section: Discussionmentioning

confidence: 99%

Neutrophil bactericidal activity and host defenses in cystic fibrosis: a narrative review

Patel,

Nugent

2023

J Thorac Dis

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Background and Objective Cystic fibrosis (CF) is a disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR). Without properly functioning CFTR channels, chloride does not exit respiratory epithelial cells, and consequently the mucus lining the surface of the cells becomes thick. This viscous mucus accumulates and causes abnormal function of the mucociliary apparatus, which can lead to bacterial colonization, infections with Staphylococcus aureus ( S. aureus ) and Pseudomonas aeruginosa ( P. aeruginosa ), and eventually lung damage. Recent studies have shown that the increased susceptibility to respiratory infections in CF patients may also be due to defects in neutrophil function, but the exact mechanism is uncertain. Methods The PubMed database was searched on February 10, 2023 and again on July 23, 2023 to compile a comprehensive list of clinical and experimental studies to evaluate neutrophil function in CF. The first search included a combination of MeSH terms: “cystic fibrosis” and “neutrophils/physiology”. A separate second search included a combination of the MeSH terms: “neutrophils” and “cystic fibrosis transmembrane conductance regulator”. Key Content and Findings Neutrophils from patients with CF have decreased transfer of chloride into phagolysosomes after bacterial ingestion and have dysregulated degranulation. This reduces the production of toxic oxidative radicals, especially hypochlorous acid (HOCl), and reduces bactericidal activity. CFTR potentiators correct the dysregulated degranulation in patients with CF and increased neutrophil killing activity. A reduced concentration of chloride in in vitro assays also reduces neutrophil killing activity; these observations are relevant to the reduced chloride concentrations in respiratory secretions in patients with CF. Conclusions This literature review summarizes studies that demonstrate that an important defect in CF neutrophils lies in the oxygen-dependent pathway in phagolysosomes and studies with ivacaftor demonstrate that this drug corrects CF neutrophil function. These studies demonstrate the potential utility of using easily available neutrophils to study drug effects in CF patients.

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Section: Discussionmentioning

confidence: 99%