Malnutrition in cystic fibrosis – beyond pancreatic insufficiency

Abstract

Malnutrition in cystic fibrosis is a result of imbalance between increased energy requirement, increased losses and poorer nutrient utilization on the one hand, and inadequate food intake on the other hand. The most important cause of maldigestion and malabsorption is pancreatic insuffi ciency. However, even optimal enzyme replacement cannot correct malnutrition as it does not target many other causes adding to the nutritional imbalance and the pathophysiological triad of infection, infl ammation and obstruction. Contributing factors are abnormal intestinal juices, dysbiosis, bile salt abnormalities and altered lipolysis, altered motility, liver disease, etc. Energy requirements are increased in comparison to healthy subjects due to chronic infection and infl ammation, and additionally rise as pulmonary function deteriorates. Energy intake may be inadequate due to gastrointestinal complications, poor nutrient utilization, or psychosocial issues. An anticipatory and active attitude of gastroenterologists and nutritionists regarding nutritional problems is mandatory even before malnutrition emerges.