Charles R. Esther Jr.
Early lung disease in children with cystic fibrosis is characterized by mucus accumulation, even in the absence of infection or structural defects, according to a study published in Science Translational Medicine. The findings suggest that agents designed to break down mucus flakes could potentially reduce the severity of early-stage symptoms.
“Our findings have two significant implications for the pediatric infectious disease specialist,” Charles R. Esther Jr., MD, PhD, professor of pediatric pulmonology at the University of North Carolina at Chapel Hill, told Infectious Diseases in Children. “First, the earliest manifestations of lung disease in [cystic fibrosis (CF)] do not reflect infection, but rather the accumulation of abnormal mucus and associated inflammation. Second, when bacteria are found in the airways of young patients with CF, they are more like anaerobic flora associated with the oral cavity rather than typical CF pathogens.”
Previous research has shown that the buildup of mucus in the lungs precedes dangerous infections and structural changes in the airway in young children with CF. However, “the mechanisms that initiate lung disease in CF have been difficult to identify because studying young subjects with CF is challenging, and CF animal models often fail to recapitulate aspects of human CF disease and yield disparate findings,” the researchers wrote.
Microscopic images compare mucus flakes from a healthy subject (left) to a flake from a patient with cystic fibrosis (right).
Source: C.R. Esther et al., Science Translational Medicine (2019)
Esther and colleagues analyzed mucus production in 46 toddlers with cystic fibrosis and 16 healthy controls. They collected samples of bronchoalveolar lavage fluid and found that children with CF had elevated levels of the protein mucin as well as an elevated density of mucus flakes in the airways.
Esther and colleagues also used computed tomography (CT) to show that children with CF who did not have bacterial infections or structural defects in the lungs still harbored increased levels of mucus flakes.
“Anti-inflammatory agents and agents designed to remove permanent mucus covering airway surfaces of young children with CF appear to be rational strategies to prevent disease progression, although these approaches likely will require development of novel therapeutics,” Esther said. – by Bruce Thiel
Disclosures: Esther reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.