Submitted to: Book Chapter
Publication Type: Book / chapter
Publication Acceptance Date: 3/12/2004
Publication Date: 6/25/2006
Citation: Cooper, A., Heird, W.C. 2006. Nutritional management of infants and children with specific diseases or other conditions. In: Shils, M.E., Shike, M., Ross, A.C., Caballero, B., Cousins, R.J., editors. Modern Nutrition in Health and Disease. 10th edition. Baltimore, MD: Lippincott Williams & Wilkins. p. 991-1003. Interpretive Summary:
Technical Abstract: Cystic fibrosis is characterized by progressive deterioration of pulmonary and pancreatic function. The former may increase nutrient requirements somewhat, but probably affects nutrition more by adversely affecting intake, particularly during acute exacerbations and in older children with severe pulmonary disease. Pancreatic insufficiency severely limits the absorption of fat, a major energy source of most diets. Thus, the cause of malnutrition in infants and children with this disease can be both primary (i.e., inadequate nutrient intake) and secondary (i.e., fecal losses of protein, and particularly, of fat). The latter cause usually can be controlled with appropriate pancreatic enzyme replacement because there does not seem to be a primary defect in energy metabolism associated with the disease.