Diet composition and quality in children with cystic fibrosis in the modulator era: A scoping review

With improved survival and changing nutritional priorities in cystic fibrosis (CF), diet quality is increasingly relevant to reduce chronic disease risks and support gastrointestinal and overall health. This scoping review aimed to identify and describe the range and characteristics of literature examining diet composition and quality in children with CF in the CF transmembrane conductance regulator (CFTR) modulator era, and identify evidence gaps to inform future research and guidelines.

Joanna Briggs Institute methodology was used. The protocol was published on Open Science Framework. Ovid MEDLINE, CINAHL and EMBASE were searched from 2012-January 2026. We included primary research studies reporting oral dietary intake in children with CF using dietary assessment methods. Two reviewers independently screened articles. Data were extracted on study characteristics, populations, dietary assessment methods, outcomes, and key findings.

Fifty-six studies were included, encompassing 4977 children with CF. Most studies examined diet composition, particularly energy and macronutrient intake; fewer assessed diet quality. Studies assessing diet quality reported high intakes of dairy, cereals and meat, low intakes of vegetables, wholegrains, legumes, nuts, eggs and fish, low dietary diversity, and low to moderate concordance with optimal diets. Only thirteen studies included children taking CFTR modulators. Younger and pancreatic sufficient children, and studies with a control group, were under-represented.

Dietary research in paediatric CF has focused on diet composition, with limited assessment of diet quality. Future research should extend beyond energy and macronutrients to better characterise diet quality and factors influencing dietary choices in children with CF.