OBJECTIVE: To review and discuss evidence on the nutritional status assessment and recommendations for nutritional management of children and adolescents with cystic fibrosis. DATA SOURCES: MEDLINE (PubMed version) and Latin American and Caribbean Center on Health Sciences Information (LILACS), from 1984 to 2009. The combination of the following keywords were applied: cystic fibrosis, nutritional status, child, and adolescent. DATA SYNTHESIS: Cystic fibrosis (CF) is a genetic disease, chronic, progressive, and fatal. It is caused by a defect in the transmembrane regulatory protein that regulates the flow of chlorine, sodium and water across the apical membrane of epithelial cells. CF patients are vulnerable to malnutrition resulting from the imbalance between food intake, energy expenditure and loss. New insights into the pathophysiology of the disease have resulted in significant changes in current recommendations for energy and especially lipids intake. There are many interrelated factors that affect the nutritional status of CF patients, such as genetic mutation, pancreatic insufficiency, bowel resection, loss of bile salts and acids, gastroesophageal reflux disease, lung inflammation and infections, diabetes, and emotional conditions. The importance of nutrition in the survival of CF patients is well established, as well as the association between malnutrition and deterioration of lung function. CONCLUSIONS: Nutritional monitoring and dietary counseling are key elements in the management of patients with CF aiming to control the symptoms and the progression of the disease in order to provide better quality of life.
cystic fibrosis; nutritional status; child; adolescent
