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RESEARCH PRODUCT

LSC Abstract – Oxidative stress in nasal epithelial cells from patients with primary ciliary dyskinesia

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Background: Primary ciliary dyskinesia (PCD) is a rare autosomal recessive inheritance disorder characterized by dysfunction of respiratory cilia and impaired mucociliary clearance, leading to respiratory problems appearing in childhood, decreased fertility and situs inversus in 50% of the cases. The deficient ciliary movement causes stasis of secretions in the airways leading to recurrent airway infection and chronic inflammation. Rationale and aims: Chronic inflammation has been associated to oxidative stress (OS). Moreover, evidence of increased OS in the airways of stable children with PCD has been shown (Zihlif, N. et al . Pediatr Pulmonol 2006; 41:509-14.). Therefore, we hypothesize that OS would be increased in ciliated nasal epithelial cells (CNEC) from patients with PCD. This study was aimed to study the OS profile in CNEC isolated from children with PCD. Materials and methods: CNEC were obtained from seven children diagnosed with PCD, six PCD- like patients and ten control individuals. OS parameters were prospectively measured by flow cytometry. Results: PCD patients showed significantly higher oxidized/reduced lipid ratio (p=0.028), H 2 O 2 (p=0.001), reduced glutathione (p=0.015), and superoxide anion (p=0.006) but lower levels of carbonylated proteins (p=0.014) than the control group. No significant differences were observed in the nitric oxide levels (p=0.373) and mitochondrial parameters between groups. Interestingly, no significant differences were observed between the PCD- like and the control groups. Conclusions: Our results show that OS is increased in CNEC of children with PCD. Funding Sources: Supported by SVN and SENP 2014 grants.