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

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Interventional

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

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 Summary:

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).


Sponsor: Assistance Publique - Hôpitaux de Paris

Current Primary Outcome: 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


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Response to amiloride during bronchial potential difference test [ Time Frame: up to 5 days ]
    Trans-epithelial transport measured in vivo
  • trans-epithelial ionic transport in the nasal epithelium (nasal DDP) [ Time Frame: up to 5 days ]
    Trans-epithelial transport measured in vivo
  • trans-epithelial ionic transport of sweat epithelium (sweat test) [ Time Frame: up to 5 days ]
    Trans-epithelial transport measured in vivo
  • Basal current short circuit [ Time Frame: up to 5 days ]
    Trans-bronchial epithelial ionic transport evaluated in vitro on bronchial primary cultures
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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


Original Secondary Outcome: Same as current

Information By: Assistance Publique - Hôpitaux de Paris

Dates:
Date Received: July 13, 2015
Date Started: April 2016
Date Completion: April 2020
Last Updated: December 2, 2016
Last Verified: December 2016