Research ArticleCystic Fibrosis

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

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Science Translational Medicine  03 Apr 2019:
Vol. 11, Issue 486, eaav3488
DOI: 10.1126/scitranslmed.aav3488

Early changes in cystic fibrosis

Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene. The lung is one of the most affected organs. Mucus accumulation, inflammation, and infection are already evident early during development. Understanding early disease pathophysiology could help the development of preventive therapies. Here, Esther et al. analyzed bronchoalveolar lavage fluid (BALF) from toddlers with CF to understand the mechanisms initiating lung disease. Abnormal mucus accumulation was evident in CF BALF, associated with increased mucin and inflammatory marker expression. These abnormalities occurred despite low bacterial infection. The results suggest that mucolytic and anti-inflammatory treatments might be effective during the early stages in CF.

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