Search results for "Primary cilia"
showing 4 items of 34 documents
High-Speed Video Microscopy for Primary Ciliary Dyskinesia Diagnosis: A Study of Ciliary Motility Variations with Time and Temperature
2021
Este artículo se encuentra disponible en la siguiente URL: https://www.mdpi.com/2075-4418/11/7/1301 Este artículo de investigación pertenece al número especial "Progress in Diagnosing and Managing Primary Ciliary Dyskinesia". Primary ciliary dyskinesia (PCD) is a rare disease resulting from a defect in ciliary function that generates, among other issues, chronic upper and lower respiratory tract infections. European guidelines recommend studying ciliary function (pattern (CBP) and frequency (CBF)), together with characteristic clinical symptoms, as one of the definitive tests. However, there is no “gold standard”. The present study aims to use high-speed video microscopy to describe how CBF…
LSC Abstract – Oxidative stress in nasal epithelial cells from patients with primary ciliary dyskinesia
2016
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 in ciliated nasal epithelial cells from patients with primary ciliary dyskinesia
2016
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 …
Limitations of nasal nitric oxide as a screening method of primary ciliary dyskinesia in childhood
2016
Introduction: Nasal nitric oxide (nNO) has been proposed as a screening tool for primary ciliary dyskinesia (PCD). However, there are no reference values by age in consensus guidelines. Objectives: 1) To analyze differences in nNO among a group of patients with PCD and healthy controls. 2) To determine the proportion of pediatric PCD patients with nNO values similar to healthy population. Methods: Determination of nNO by electrochemical system (NIOX Vario®) in patients with DCP (diagnosis confirmed by digital high speed videomicroscopy) and healthy controls (without asthma, rhinitis or atopic dermatitis), aged between 7 and 60 years. Results: 32 patients (17 children, 53.1%) and 32 controls…