Abstract

Malnutrition is recognised as a major prognostic factor adversely affecting survival in cystic fibrosis (CF) and is the result of an unfavourable energy balance in these patients. A high resting energy expenditure, dependent on the patient's genotype, in addition to pulmonary infection play an important role in producing anorexia and weight loss. Nutritional management with the aim to gain weight and catch up with growth is age-specific. It is important to repair nutritional status as early as possible after diagnosis. In infancy breast feeding is advised with, if necessary, supplemental feeding with predigested formulae such as Pregestimil. In childhood nutritional management must be aimed towards a normal weight gain and growth velocity. The latter is the best guide of nutritional adequacy. If weight gain falters the first principle is to treat any associated respiratory infection, the second is to ensure adequate enzyme therapy and control of steatorrhoea, and only then should dietary energy supplements be introduced. When oral hypernutrition fails, nocturnal naso-gastric tube feeding of a non-elemental formula may be considered. Parenteral nutrition is rarely indicated and should be reserved as a last solution for CF-patients.