Aided by a new experimental model, scientists are a step closer to understanding how cystic fibrosis (CF) causes lung disease in people with the condition.
The findings, published online April 28 in the journal Science Translational Medicine, could help improve treatments for lung disease, which causes most of the deaths and disability among people with CF.
In particular, the study by a University of Iowa research team and colleagues at University of Missouri appears to answer a long-standing "chicken and egg" question about which comes first in CF lungs -- infection or inflammation.
"Using our model we are beginning to answer that question, and it looks like infection does precede inflammation," said David Stoltz, M.D., Ph.D., UI assistant professor of internal medicine and lead study author. "The importance of that finding is that it could dictate what types of therapy we might use. Knowing that infection is first suggests that if we can prevent or fight infection, then that might delay or prevent the lung disease in people with CF."
For example, the finding would seem to support early and aggressive treatment of lung infections in children with CF, added Stoltz, who also is assistant director of the Adult Cystic Fibrosis Center at UI Hospitals and Clinics.
The new experimental model that Stoltz and his colleagues used were pigs with a CF-causing gene mutation. The team generated the pig model in the hope that it would more closely mimic the human disease than mouse models do.