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Ion transport in airways: cystic fibrosis and neonatal respiratory distress

Document type: Projects: In progress/completed
Document language: English
Uploaded By: Karl Gauffin (kgauf)
Date Uploaded: 2012/02/03 10:50:54.343 GMT+1
URL to a webpage or document based online: http://

Basic Information

Authors: Godfried Roomans
Funding source project: Swedish Research Council

The project comprises two areas, cystic fibrosis, and neonatal respiratory distress. These conditions have overlaps (epithelial Na+ and Cl- transport, innate immunity). Cystic fibrosis (CF) is due to a defect in a Cl- channel (CFTR). We investigate ways to correct this defect, by overcoming intracellular breakdown of CFTR, or by activating alternative (Ca2+-dependent) Cl- channels. We have obtained positive results with S-nitrosoglutathione, N-acetylcysteine and azithromycin and plan to further test these and other compounds (miglustat, ambroxol, PPARg). We have found a coupling between CFTR and tight junctions, which could affect water transport, and will continue these studies. We have shown that the salt concentration in the airway surface liquid of CF-patients is abnormally high, which could affect parts of the innate immune system, and preliminary data indicate a defect in this system in CF cells. Neonatal respiratory distress is a common clinical problem. At birth, mechanisms for ion/water transport in the airways change from one dominated by Cl- and water efflux to one dominated by Na+ and water influx. Problems in this change result in transient tachypnoea of the newborn (TTN) and respiratory distress syndrome (RDS) in premature children. At present knowledge about ion/water transport in the lungs around birth is lacking, and studies on Na+ and Cl- transport in the airway epithelium will help to develop treatment modes for neonatal respiratory distress.

Riche Classification

Demography: Genetic studies , Perinatal: , Neonatal: ,
Health issues, determinants and measures: Morbidity , Respiratory disorders , Analytical, Diagnostic and therapeutic Techniques and Equipment , Medicines , Respiratory ,
Study type / scale / state of progress / setting: Health surveillance , Evaluation research - of policy or of practice, interventions. ,