By Tim Bonfield
The Cincinnati Enquirer
CORRYVILLE - Researchers at Cincinnati Children's Hospital Medical Center and the University of Southern California have identified a new way to attack cystic fibrosis, one of the most common and still uncured genetic diseases.
The idea is to develop a drug to fight the prolonged inflammation that causes tissues to swell and trap bacteria in the airways of children, which in turn can lead to life-threatening infections.
According to a study published Sunday in the journal Nature Immunology, children with cystic fibrosis have trouble producing a class of molecules called lipoxins that help regulate inflammation.
About 30,000 Americans have cystic fibrosis, a disease that causes abnormally thick, sticky mucus, chronic lung infections and impaired digestion.
With improved treatments in recent years, many people with the disease survive into their early 30s.
Cincinnati Children's has several researchers studying the disease, and it has received millions in public and private research grants - including support from former Cincinnati Bengals player Boomer Esiason, whose son, Gunnar, has cystic fibrosis.
The most recent study found that mice - genetically engineered to develop cystic fibrosis - had a better inflammation response when given lipoxin-like molecules. That suggests such molecules might have therapeutic potential for people with cystic fibrosis, said Dr. Christopher Karp, director of molecular immunology at Cincinnati Children's and the study's main author.
The study was co-authored by Dr. Nicos Petasis of USC.
"It has traditionally been thought that the basic problem in the CF lung is an inability to clear bacteria, with infection leading secondarily to lung-damaging inflammation," Karp said. "Recent studies suggest it may well be the other way around. It's clear that the inflammatory response of the airway in CF patients is abnormal from the earliest time you look at it."
The Children's Hospital study was sponsored by an affiliate of the Cystic Fibrosis Foundation.
If a safe drug can be developed, controlling inflammation could help increase survival times for people with cystic fibrosis and reduce the demand for lung transplantation, according to a statement from Dr. Robert Beall, president and chief executive of the Cystic Fibrosis Foundation.
"The more we know about the unique cycle of inflammation in CF, the better we are able to develop the appropriate tools to fight it," Beall said.
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E-mail tbonfield@enquirer.com
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