CURRENT CONCEPTS OF NUTRITIONAL THERAPY I N CYSTIC-FIBROSIS

Patients with cystic fibrosis (CF) frequently develop malnutrition, wh ich is associated with poor pulmonary function, increased incidence of infection and reduced survival rates. Malnutrition results from a dis crepancy between high energy demands, primarily due to an increased wo rk of breathing in patients with limited pulmonary function, and low e nergy uptake due to malabsorption and inaedaquate food consumption. In addition to an energy deficit, deficiency of individual nutrients may develop. Symptomatic protein deficiency occurs mainly in infants and in older CF patients with severe malnutrition. Salt deficiency with de hydration or hypochloraemic alkalosis is found primarily in infancy an d is associated with increased sweat loss. The availability of water s oluble vitamins is usually not compromised, with the possible exceptio n of vitamin B-12. In contrast, patients with steatorrhea frequently d evelop a deficiency of lipid soluble vitamins, therefore, prophylactic supplementation is required in all CF patients with exocrine pancreat ic insufficiency. Essential fatty acid status is also poor in many pat ients. Nutritional therapy in CF follows a stepwise concept and is ada pted to the individual patient. From the time of diagnosis, regular mo nitoring of nutritional status and repeated dietary counseling is mand atory. Subnormal weight gain should prompt early intervention with int ensified dietary counseling aiming at increased energy intake with hom e made foods. If the weight response is not adaequate, the use of supp lemental formula and possibly also of nighttime or continuous gavage f eeding should be considered.