Inhalation therapy proves effective against cystic fibrosis
February 6th, 2009 - 5:48 pm ICT by ANIWashington, Feb 6 (ANI): By spraying amiloride into the lungs of young mice, scientists have successfully prevented the onset of cystic fibrosis lung disease in mice.
The researchers at Department of Paediatrics at Heidelberg University Hospital have demonstrated for the first time that preventative therapy for the lung disease is possible.
Attacking the root cause of the widespread hereditary disease, the mice in the study were given an inhalation treatment with the drug in the first days of life.
It was found that no thick mucus forms in the lungs and airway inflammation and chronic lung damage could be prevented.
Cystic fibrosis (CF) or mucosviscidosis or is the most common life-shortening genetic disease, caused by defects at a certain locations in the genetic makeup a mutation in what is called the CFTR (”Cystic Fibrosis Transmembrane Conductance Regulator” gene).
The mutation leads to loss of salt and water and thus dehydration of the surfaces of the mucous membranes in the lungs, intestines, and other organs.
Amiloride inhibits hyperactive sodium channels and prevents the mucous membranes from drying out
Using the mouse model he developed, Dr. Marcus Mall, physician scientist and head of the Cystic Fibrosis Center at the Department of Pediatrics has shown that certain hyperactive sodium channels in airway cells are responsible for the increased absorption of salt and water from airway surfaces.
For the study, researchers tested whether inhibiting these hyperactive sodium channels with amiloride could improve hydration of the airway surfaces and prevent lung damage.
It was found that in a mouse model, amiloride administered in the first few days of life prevents the typical symptoms of cystic fibrosis and the development of chronic lung disease.
However, the treatment brought no improvement if the symptoms were already present
Chronic lung damage apparently prevents the amiloride from being effective.
This indicates that the lung damage caused by the disease may be irreversible, stated Dr. Mall.
With amiloride, preventative therapy of cystic fibrosis that attacks the basic defect directly could be possible for the first time. But the children affected would have to be identified at a very early stage, said Dr. Mall.
The study has been published in the American Journal of Respiratory and Critical Care Medicine. (ANI)
- Novel drug may benefit cystic fibrosis patients - Dec 18, 2010
- Protein involved in cystic fibrosis linked to chronic lung diseases - Dec 30, 2010
- Sodium channel blocker may benefit cystic fibrosis patients - May 18, 2009
- Scientists one step closer to a drug treatment for cystic fibrosis - Oct 13, 2010
- Boffins come closer to understanding how cystic fibrosis causes lung disease - Apr 29, 2010
- Crosstalk between ion channels leads to new therapeutic strategy - Sep 20, 2010
- Dry airways play key role in development of chronic lung diseases - Apr 08, 2008
- Infliximab treatment effective for cystic fibrosis and Crohn's disease - Apr 20, 2010
- New therapeutic target for asthma, other lung disorders identified - Apr 18, 2011
- Drug targets defective protein that causes cystic fibrosis - Nov 18, 2010
- Enzyme essential for healthy lung development discovered - Mar 30, 2011
- Novel finding could lead to new therapies for asthma, COPD - Jan 28, 2011
- Cigarette smoke may cause inflammation through a new chemical pathway - Sep 03, 2010
- Vitamin D deficiency alters lung structure and function - Jan 29, 2011
- Growth defects in patients with cystic fibrosis 'may start before birth' - Nov 10, 2010
Tags: airway inflammation, amiloride, chronic lung disease, cystic fibrosis, cystic fibrosis center, cystic fibrosis transmembrane conductance regulator, first few days, genetic makeup, heidelberg university, hereditary disease, inhalation therapy, lung damage, mouse model, physician scientist, preventative therapy, regulator gene, salt and water, sodium channels, symptoms of cystic fibrosis, thick mucus
