'First step' discovery could offer new hope to CF sufferers
People with cystic fibrosis may benefit from a new discovery by Irish researchers which could potentially reduce the need for lung transplants and lower their risk of death.
The "first step" discovery by researchers at the Royal College of Surgeons in Ireland (RCSI) could pave the way for treatment over time to reduce inflammation in people with the disease which affects 1,300 children and adults in Ireland.
The researchers found that one of the most aggressive bacteria found in the lungs of those with cystic fibrosis caused certain immune cells to change their metabolism.
This led to the immune cells producing a protein that causes more inflammation.
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High levels of the protein were associated with lower lung function and a higher risk of death or need for a lung transplant.
"This is an important first step to significantly improving patient outcomes for people with cystic fibrosis," said Professor Gerry McElvaney, the study's joint senior author and professor of medicine at RCSI.
"While more testing is required before delivering this to patients, we believe these results are very promising and could make this molecule a candidate for clinical trials."
The team used a small molecule called MCC950 to reduce levels of the protein in a laboratory model of cystic fibrosis. Along with lowering inflammation it helped clear the lungs of bacteria.
"Previously, people with CF had a very low life expectancy," said Dr Oliver McElvaney, the study's lead author.
"Due to improvements in medical treatment, these individuals are now living longer.
"However, they still suffer from a very severe disease. We hope that this advancement can lead to further improvements in outcome, better quality of life and eventually a normal life expectancy for our patients."
The study, which is in collaboration with the University of Duisburg-Essen and the Trinity Biomedical Sciences Institute, is published in the current edition of the 'American Journal of Respiratory and Critical Care Medicine'.