Lindsay J. Caverly, Benjamin D. Ross, Robert P. Dickson
European Respiratory Journal 2024 63: 2400595; DOI: 10.1183/13993003.00595-2024 

Extract

The connection between gastrointestinal and respiratory pathology in cystic fibrosis (CF) was first observed in 1938 in Dorothy Andersen's initial description of CF [1]. Andersen reported on the autopsy findings of 49 infants and young children with CF, which notably included near-universal evidence of pancreatic fibrosis, nutritional deficiencies and chronic respiratory infections. In the following decades, dramatic increases in life expectancy for people with CF largely resulted from therapies directed towards gastrointestinal and respiratory disease, including pancreatic enzymes, calorie and vitamin supplementation, and antibiotics. Multiple observational studies have demonstrated links between nutrition and respiratory outcomes, particularly in the first years of life. For example, higher weight- and height-for-age in infancy and toddlerhood has been associated with improved respiratory outcomes in later childhood and adolescence, including better lung function and lower rates of respiratory infection with Pseudomonas aeruginosa [2, 3].