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

Primary Purpose

Idiopathic Dilation of the Bronchi

Status
Completed
Phase
Not Applicable
Locations
France
Study Type
Interventional
Intervention
bronchial ddp test
Sponsored by
Assistance Publique - Hôpitaux de Paris
About
Eligibility
Locations
Arms
Outcomes
Full info

About this trial

This is an interventional diagnostic trial for Idiopathic Dilation of the Bronchi focused on measuring Dilation of the Bronchi, Bronchial potential difference, Dilation of the Bronchi (DDB)

Eligibility Criteria

2 Years - 20 Years (Child, Adult)All SexesDoes not accept healthy volunteers

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.

Sites / Locations

  • Necker-Enfants Malades Hospital

Arms of the Study

Arm 1

Arm 2

Arm 3

Arm Type

Experimental

Active Comparator

Active Comparator

Arm Label

Patients with multiple bronchi dilations

Control patients without transport abnormality

Patients with typical cystic fibrosis

Arm Description

Outcomes

Primary Outcome Measures

Response to isoproterenol in solution without chloride ions during bronchial potential difference test (ΔIsoprotérénol / bronchial).
Difference between groups for Level of repolarisation

Secondary Outcome Measures

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

Full Information

First Posted
July 13, 2015
Last Updated
April 16, 2021
Sponsor
Assistance Publique - Hôpitaux de Paris
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1. Study Identification

Unique Protocol Identification Number
NCT02586883
Brief Title
Bronchial Trans-epithelial Transport in Patients With Idiopathic Multiple Dilations of the Bronchi
Acronym
EPITRANS
Official Title
Study of the Bronchial Trans-epithelial Transport in Patients With Idiopathic Multiple Dilations of the Bronchi
Study Type
Interventional

2. Study Status

Record Verification Date
April 2021
Overall Recruitment Status
Completed
Study Start Date
March 29, 2016 (Actual)
Primary Completion Date
November 23, 2020 (Actual)
Study Completion Date
November 23, 2020 (Actual)

3. Sponsor/Collaborators

Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Assistance Publique - Hôpitaux de Paris

4. Oversight

Data Monitoring Committee
No

5. Study Description

Brief Summary
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.
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).

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Idiopathic Dilation of the Bronchi
Keywords
Dilation of the Bronchi, Bronchial potential difference, Dilation of the Bronchi (DDB)

7. Study Design

Primary Purpose
Diagnostic
Study Phase
Not Applicable
Interventional Study Model
Parallel Assignment
Masking
None (Open Label)
Allocation
Non-Randomized
Enrollment
42 (Actual)

8. Arms, Groups, and Interventions

Arm Title
Patients with multiple bronchi dilations
Arm Type
Experimental
Arm Title
Control patients without transport abnormality
Arm Type
Active Comparator
Arm Title
Patients with typical cystic fibrosis
Arm Type
Active Comparator
Intervention Type
Other
Intervention Name(s)
bronchial ddp test
Intervention Description
bronchial ddp test during endoscopy, nasal smear, bronchial smear
Primary Outcome Measure Information:
Title
Response to isoproterenol in solution without chloride ions during bronchial potential difference test (ΔIsoprotérénol / bronchial).
Description
Difference between groups for Level of repolarisation
Time Frame
up to 5 days
Secondary Outcome Measure Information:
Title
Response to amiloride during bronchial potential difference test
Description
Trans-epithelial transport measured in vivo
Time Frame
up to 5 days
Title
trans-epithelial ionic transport in the nasal epithelium (nasal DDP)
Description
Trans-epithelial transport measured in vivo
Time Frame
up to 5 days
Title
trans-epithelial ionic transport of sweat epithelium (sweat test)
Description
Trans-epithelial transport measured in vivo
Time Frame
up to 5 days
Title
Basal current short circuit
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Response to Epithelial Sodium Channel (ENaC) inhibitors
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) (inh-172)
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Activation of Calcium (Ca)-dependent Chloride (Cl-) ion channels
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Inhibition of Solute Carrier family 26, member 9 (SLC26A9)
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Response to inhibitors of potassium (K+ ) secretion basolateral
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days
Title
Bicarbonate (HCO3- ) secretion in response to forskolin
Description
Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
Time Frame
up to 5 days

10. Eligibility

Sex
All
Minimum Age & Unit of Time
2 Years
Maximum Age & Unit of Time
20 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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.
Facility Information:
Facility Name
Necker-Enfants Malades Hospital
City
Paris
ZIP/Postal Code
75015
Country
France

12. IPD Sharing Statement

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

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