Functional Analysis by Dynamic Imaging of the Respiratory Epithelium in Infants With Cystic Fibrosis

NCT01605565 | Completed

• 2 other identifiers: C11-012011-A00384-37
• Study Type: interventional
• Target: 30
• Locations: 1 country
• Sites: 1

Brief Summary

Cystic fibrosis (CF) is characterized by airway inflammation and infection leading to progressive destruction of lungs. One of the most important abnormalities in CF is an abnormal processing of the mutated CFTR protein through the endoplasmic reticulum that causes abnormal location or even absence of the protein at the apical plasma membrane of airway epithelial cells. This abnormality results in a marked dehydration of the airway surface fluid, decreased mucus transport and airway obstruction. Nevertheless, the events that occur very early during the progression of the disease at the airway level in infants are not known. At cellular level, it has also been reported that the CFTR expression and localization could be related to the differentiation state of the airway epithelium. Furthermore, it has been reported that gap junctions could be involved in dysregulate inflammation process. In CF infants, many answers are still lacking. For a better understanding of the early stages of cystic fibrosis, it is of major interest to study respiratory epithelial cells obtained as early as possible. In 15 CF infants and 15 control infants, a nasal brushing will be performed by means of a soft sterile cytology brush. Samples will be used for cytological and functional studies: ciliary beating frequency, cAMP-dependent chloride efflux, potassium efflux, tight and gap junctions functionalities. These studies will be done in basal conditions and will be repeated after activation of the nasal epithelial cells by the bacteria, such as Staphylococcus aureus, which can be found very early in the course of CF disease.

Conditions

Cystic Fibrosis

Outcome Measures

Primary Outcomes (1)

• The main objective is to analyze the functionality of the respiratory epithelium in CF infant using a nasal brushing technique: ciliary beating frequency, cAMP-dependent chloride efflux, potassium efflux, tight and gap junctions functionalities.

  In 15 CF infants and 15 control infants, a nasal brushing will be performed by means of a soft sterile cytology brush. Samples will be used for cytological and functional studies: ciliary beating frequency, cAMP-dependent chloride efflux, potassium efflux, tight and gap junctions functionalities.

  no time frame

Interventions

A nasal brushing PROCEDURE

A nasal brushing in every nostril

Eligibility Criteria

• Age: 1 Week - 6 Months
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17)

You may qualify if:

• Cystic fibrosis

You may not qualify if:

• \> 6 months old
• Other respiratory disease
• Other allergic disease
• Other infectious disease: fever (\> 38° C), respiratory distress
• Altered general health state, rash,

Sponsors & Collaborators

• Institut National de la Santé Et de la Recherche Médicale, France: lead

Study Sites (1)

Chu Reims

Reims, 51092, France

Location

Related Publications (1)

• Mosler K, Coraux C, Fragaki K, Zahm JM, Bajolet O, Bessaci-Kabouya K, Puchelle E, Abely M, Mauran P. Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants. J Cyst Fibros. 2008 Jan;7(1):44-53. doi: 10.1016/j.jcf.2007.04.005. Epub 2007 Jun 5.

  PMID: 17553758 BACKGROUND

MeSH Terms

Conditions

Cystic Fibrosis

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases

Study Officials

• Michel ABELY, Professor

  CHU REIMS

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: PARALLEL
• Sponsor Type: OTHER GOV
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 11, 2012
• First Posted: May 25, 2012
• Study Start: November 1, 2011
• Primary Completion: September 18, 2013
• Study Completion: September 18, 2013
• Last Updated: January 23, 2025

Record last verified: 2025-01

Locations

FR (1)