0000000000082827
AUTHOR
Sara Pastor
Oxidative stress in ciliated nasal epithelial cells from patients with primary ciliary dyskinesia
Background: Primary ciliary dyskinesia (PCD) is a rare disorder characterized by dysfunction of respiratory cilia and impaired mucociliary clearance leading to recurrent airway infection and chronic inflammation appearing in childhood. 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). We hypothesize that OS would be increased in ciliated nasal epithelial cells (CNEC) from patients with PCD. This study was aimed to assess the OS profile in CNEC isolated from children with PCD. Methods: CNEC were obtained from …
LSC Abstract – Increased oxidative stress leads to telomere shortening in children with alpha-1 antitrypsin deficiency
Background: Oxidative stress (OS) is involved in the pathophysiology of AATD (Escribano A. et al. Thorax 2015; 70:82-3). In addition, it has been shown that OS accelerates telomere shortening which is associated to higher emphysema risk in COPD patients. Rationale and aims: 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. This study is aimed to assess the OS profile, the enzymatic antioxidant defence mechanisms and telomere length (TL) in children with AATD and to study its association with AAT phenotypes. Methods: OS parameters, the activity of the ma…
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…
Decreased glutathione and low catalase activity contribute to oxidative stress in children with α-1 antitrypsin deficiency: Table 1
Background Recent investigations in animal models have revealed oxidative stress and oxidative damage in the pathogenesis of alpha-1 antitrypsin deficiency (AATD). However, no data are available on the oxidative stress status and antioxidant enzyme activity in these patients. This study was aimed to analyse the oxidative stress profile and enzymatic antioxidant defence mechanisms in children with AATD. Methods Oxidative stress parameters and the activity of the main antioxidant enzymes were prospectively measured in serum of fifty-one children diagnosed with AATD and thirty-eight control individuals. Results Oxidative stress was increased in the serum of children with intermediate- (MZ; SZ)…
Oxidative Stress and Endoplasmic Reticulum Stress in Rare Respiratory Diseases
Several studies have shown that some rare respiratory diseases, such as alpha-1 antitrypsin deficiency (AATD), idiopathic pulmonary fibrosis (IPF), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD) present oxidative stress (OS) and endoplasmic reticulum (ER) stress. Their involvement in these pathologies and the use of antioxidants as therapeutic agents to minimize the effects of OS are discussed in this review.
Plasma circulating miRNAs as diagnostic and prognostic biomarkers in alpha-1 antitrypsin deficiency
Introduction: Alpha-1 antitrypsin (AATD) deficiency is an inherited condition that leads to decreased circulating AAT levels, significantly increasing the risk of lung and liver disease. AATD is underdiagnosed. Severity of symptoms in AATD patients are highly variable and neither protein levels nor phenotype are sufficient to identify which patients will develop lung and/or liver disease. Therefore, new strategies and biomarkers for early diagnosis and prognosis of the disease are needed. Rationale and Aims: MicroRNAs (miRNAs) regulate gene expression and have been associated with the pathogenesis of various lung and liver diseases. Circulating miRNAs may serve as diagnostic and prognostic …
Accelerated telomere attrition in children and teenagers with α1-antitrypsin deficiency.
Numerous studies have shown that oxidative stress accelerates telomere shortening in several lung pathologies. Since oxidative stress is involved in the pathophysiology of α1-antitrypsin deficiency (AATD), we hypothesised that telomere shortening would be accelerated in AATD patients. This study aimed to assess telomere length in AATD patients and to study its association with α1-antitrypsin phenotypes.Telomere length, telomerase activity, telomerase reverse transcriptase (hTERT) expression and biomarkers of oxidative stress were measured in 62 children and teenagers (aged 2–18 years) diagnosed with AATD and 18 controls (aged 3–16 years).Our results show that intermediate-risk (MZ; SZ) and …
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…
Oxidative Stress Parameters in Saliva and Its Association with Periodontal Disease and Types of Bacteria
Objective. To determine the association between oxidative stress parameters with periodontal disease, bleeding, and the presence of different periodontal bacteria.Methods. A cross-sectional study in a sample of eighty-six patients, divided into three groups depending on their periodontal status. Thirty-three with chronic periodontitis, sixteen with gingivitis, and thirty-seven with periodontal healthy as control. Oxidative stress biomarkers (8-OHdG and MDA), total antioxidant capacity (TAOC), and the activity of two antioxidant enzymes (GPx and SOD) were determined in saliva. Subgingival plaque samples were obtained from the deepest periodontal pocket and PCR was used to determine the prese…