Nutritional challenges of infants with cystic fibrosis.

Nutrition plays an essential role in the management of cystic fibrosis (CF), particularly in infants with their high energy requirement due to rapid growth. Most infants are already malnourished at the time of clinical diagnosis due to an energy imbalance with increased losses and needs not compensated by energy intake. Even in very young asymptomatic CF patients diagnosed by neonatal screening programs, nutritional deficits can be detected in a high proportion of infants, since about half of all CF patients have evidence of pancreatic insufficiency at the time of birth. This review provides recommendations for assessment of the nutritional status at diagnosis and nutritional management including supplementation with pancreatic enzymes during the first year of life.