Abstract
<jats:p>Cystic fibrosis (CF) is one of the most common recessively inherited life‐shortening diseases, affecting one in 3500 white births in Europe. Advances in both pulmonary and nutritional management have helped to improve clinical and nutritional outcomes and, in recent years, the focus has shifted from that of survival to longevity. Children with CF in the United Kingdom receive either full care from a specialist centre or shared care with a designated network centre. All patients have an annual assessment, which includes a range of investigations and a full dietary assessment. Assessment of nutritional status includes regular monitoring of anthropometry, with an increasing, additional focus on body composition. Traditionally, nutritional treatments have targeted symptoms of cystic fibrosis transmembrane conductance regulator dysfunction to control malabsorption and correct undernutrition. Measures include pancreatic enzyme replacement therapy, fat‐soluble vitamin and mineral supplementation, and where needed, a high‐fat, high‐energy diet and oral or enteral nutrition support.</jats:p>