Bronchial Trans-epithelial Transport in Patients With Idiopathic Multiple Dilations of the Bronchi (EPITRANS)

April 16, 2021 updated by: Assistance Publique - Hôpitaux de Paris

Study of the Bronchial Trans-epithelial Transport in Patients With Idiopathic Multiple Dilations of the Bronchi

The purpose of this study is to identify the abnormalities of bronchial trans-epithelial transport of chloride, sodium and bicarbonate in patients with idiopathic dilations of the bronchi.

Study Overview

Status

Completed

Intervention / Treatment

Detailed Description

Dilations of the bronchi happens for one child over 3000. The extended forms may progress to respiratory failure. More than one case over two remains of undetermined cause. If the prototype is the cystic fibrosis, other abnormalities of ionic transport may be the cause of a failure of mucociliary clearance and enhance the idiopathic dilations of the bronchi.

The purpose of this study is to identify the abnormalities of bronchial trans-epithelial transport of chloride, sodium and bicarbonate in patients with idiopathic dilations of the bronchi, in comparison to two others groups of patients (without abnormality of ionic transport/with typical cystic fibrosis).

Study Type

Interventional

Enrollment (Actual)

42

Phase

  • Not Applicable

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

      • Paris, France, 75015
        • Necker-Enfants Malades Hospital

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

2 years to 20 years (Child, Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Description

Inclusion Criteria:

Common criteria for all patients

  • Age between 2 and 20 years.
  • Patient weighing more than 12kg
  • Patients with a scheduled bronchoscopy under clinical monitoring (assessment of bronchial involvement, local samples for bacteriological and histological examination)
  • Signature of consent by the patient or by the / the holder (s) of parental authority and the investigator
  • Patient affiliated to a social security scheme or entitled
  • Patient with contraception (for woman of childbearing age) Specific criteria for idiopathic bronchiectasis patients Patient with idiopathic bronchiectasis in at least two lobes, diagnosis made after extensive screening of known acquired or congenital causes

Specific criteria for "control" patients without abnormal ion transport

  • Patient Not having bronchiectasis s or any supposed alteration in transepithelial ion transport
  • Patients with fiberoptic bronchoscopy performed for one of the following indications:
  • Pulmonary malformations
  • Laryngeal, tracheal, bronchomalacia
  • Airway compression
  • Interstitial pathology
  • Suspicion of foreign body
  • Suspected tuberculosis Specific criteria for patients with a typical form of cystic f ibrosis (CF) Patient carrying 2 causing mutations in the CFTR gene (according to CFTR2 database; http://www.cftr2.org/mutations_history.php) and sweat test> 60 milliequivalent per liter (mEq/L).

Exclusion Criteria:

Common criteria for all patients

  • Smoking passive or active
  • Not essential bronchial endoscopy in the clinical follow
  • Extension of bronchoscopy time attributed to the difference in potential bronchial incompatible with the patient's general status
  • Patient pregnant or breast feeding
  • Hypersensitivity or cons known contraindications to health products for measurement of DDP (isoproterenol, Amiloride, ATP)

Specific criteria for idiopathic DDB patients

Presence of other congenital or acquired etiologies of DDB:

  • Typical or atypical cystic fibrosis,
  • Immunodeficiency,
  • Primary ciliary dyskinesia,
  • Abnormal bronchial wall structure (Williams-Campbell syndrome, Mounier-Kuhn, Ehlers-Danlos syndrome, Marfan's disease)
  • Infectious DDBs post
  • Extrinsic or endobronchial obstruction (foreign body, malformation, middle lobe syndrome)
  • Chronic inhalation (GERD, swallowing disorders, gastroesophageal tracheal fistula)
  • Allergic bronchopulmonary aspergillosis,
  • System disease.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Diagnostic
  • Allocation: Non-Randomized
  • Interventional Model: Parallel Assignment
  • Masking: None (Open Label)

Arms and Interventions

Participant Group / Arm
Intervention / Treatment
Experimental: Patients with multiple bronchi dilations
bronchial ddp test during endoscopy, nasal smear, bronchial smear
Active Comparator: Control patients without transport abnormality
bronchial ddp test during endoscopy, nasal smear, bronchial smear
Active Comparator: Patients with typical cystic fibrosis
bronchial ddp test during endoscopy, nasal smear, bronchial smear

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Response to isoproterenol in solution without chloride ions during bronchial potential difference test (ΔIsoprotérénol / bronchial).
Time Frame: up to 5 days
Difference between groups for Level of repolarisation
up to 5 days

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Response to amiloride during bronchial potential difference test
Time Frame: up to 5 days
Trans-epithelial transport measured in vivo
up to 5 days
trans-epithelial ionic transport in the nasal epithelium (nasal DDP)
Time Frame: up to 5 days
Trans-epithelial transport measured in vivo
up to 5 days
trans-epithelial ionic transport of sweat epithelium (sweat test)
Time Frame: up to 5 days
Trans-epithelial transport measured in vivo
up to 5 days
Basal current short circuit
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Response to Epithelial Sodium Channel (ENaC) inhibitors
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) (inh-172)
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Activation of Calcium (Ca)-dependent Chloride (Cl-) ion channels
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Inhibition of Solute Carrier family 26, member 9 (SLC26A9)
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Response to inhibitors of potassium (K+ ) secretion basolateral
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days
Bicarbonate (HCO3- ) secretion in response to forskolin
Time Frame: up to 5 days
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
up to 5 days

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

March 29, 2016

Primary Completion (Actual)

November 23, 2020

Study Completion (Actual)

November 23, 2020

Study Registration Dates

First Submitted

July 13, 2015

First Submitted That Met QC Criteria

October 23, 2015

First Posted (Estimate)

October 27, 2015

Study Record Updates

Last Update Posted (Actual)

April 19, 2021

Last Update Submitted That Met QC Criteria

April 16, 2021

Last Verified

April 1, 2021

More Information

Terms related to this study

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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