Nutritional considerations in patients with cystic fibrosis

Abstract

Nutritional status is strongly associated with pulmonary function and survival in cystic fibrosis patients. Attainment of a normal growth pattern in childhood and maintenance of adequate nutritional status in adulthood represent major goals of multidisciplinary cystic fibrosis centers. International guidelines on energy intake requirements, pancreatic enzyme-replacement therapy and fat-soluble vitamin supplementation are of utmost importance in daily practice. The present review summarizes the most up-to-date information on early nutritional management in newly diagnosed patients and evaluates the benefits of aggressive nutritional support, assessment of nutritional status, recommendations for nutrition-related management in pancreatic-insufficient patients and the possible therapeutic impact of fat intake modulation upon inflammatory status.