0000000000411967

AUTHOR

Shirley Camacho

Telomere attrition in peripheral blood mononuclear cells of children with alpha-1 antitrypsin deficiency

Background: Our research group have demonstrated that oxidative stress (OS) is involved in the pathophysiology of alpha-1 antitrypsin deficiency (AATD) (Escribano A. et al. Thorax 2015;70:82-3). In addition, many evidences have shown that OS accelerates telomere shortening in several lung pathologies. Short telomeres have been associated to higher emphysema risk in COPD patients. Rationale: Since AATD is characterised by chronic OS, we hypothesise that telomere shortening would be accelerated in AATD patients and would be associated with higher risk of developing lung disease. Aims: To assess telomere length (TL) in AATD patients and to study its association with AAT phenotypes. Methods: TL…

research product

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

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 t…

research product