**17. Nutritional status and weight in determining outcome in cystic fibrosis**

The impact of malnutrition in CF is of great interest; exocrine and endocrine dysfunction, coupled with high basal metabolic requirements in the setting of chronic disease, exposes patients to a high risk of malnourishment. Low BMI in CF is known to increase in incidence with age and be closely implicated in worsening lung function [91, 92] and predicts worse outcome over a 4-year period [93]. Furthermore, patients awaiting lung transplantation who require nutritional intervention have been shown to have a higher risk of death [86] and improving the nutritional status of malnourished patients may ultimately improve lung function, even in cohorts with relatively advanced lung disease [94-96].
