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The majority of individuals with cystic fibrosis (CF), primarily those with CF transmembrane regulator (CFTR) class I, II or III mutations, have pancreatic insufficiency and protein/fat malabsorption requiring nutritional management and pancreatic enzyme replacement therapy.1 While the focus of CF treatment over the last 50 years has been on lung disease, the relationship between nutrition (as reflected by weight) and life expectancy in people with CF has long been appreciated. Infants who achieve a weight z-score at 2 years of age that is at or above their birth weight z-score have significantly better lung function at age 6,2 and low weight for age and weight for length during early childhood is a risk factor for early mortality.3 4 Implementation of CF newborn screening, along with high-calorie nutrition interventions and pancreatic enzyme replacement, has led to significantly improved growth parameters in children with CF over the last few decades.5 6
Malabsorption, in combination with increased metabolic expenditures related to lung disease, is the most obvious cause of poor growth in CF. Nutritional interventions have been able to achieve a relative normalisation of weight but have been less successful at preventing or reversing stunting. Only 32% of children diagnosed by newborn screening in the Wisconsin Randomized Clinical Trial achieved target mid-parental height by puberty7; in 2012, the year of that analysis, 54% of children 2–19 years of age in the CF Foundation national registry were above the 50th percentile for body mass index.8 Infants participating in the Baby Observational and Nutritional Study showed an increase in their weight z-score, but a decrease in length z-score during their first year of life, and nearly twice as many (24%) were below the 10th percentile for length than were below the 10th percentile for weight (24%). It appears …
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