Nutritional management in cystic fibrosis

Abstract

Cystic fibrosis (CF) is the most common hereditary fatal disease but the survival rate has improved markedly over the past 30 years. The three main reasons include specialised knowledge by centralisation of medical treatment, improved treatment of the bacterial infections in the lungs and the increased awareness of the importance of nutrition. Different factors, which affect the nutritional status have to be considered when nutritional recommendations in cystic fibrosis are given. These factors are abnormal metabolism, malabsorption and a decreased intake. An ordinary diet with a high mixed fat content including essential fatty acids together with supplement of fat soluble vitamins in water dispersion, are the basic in the nutritional treatment of the disease.

Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?

“…Interestingly, the sodium excretion improved and even normalized in some cases after three years of LA supplementation, and also the glomerulus filtration normalized [ 80 ]. An investigation of total body sodium homeostasis with 24 Na did not indicate a high sodium loss in the patients [ 88 ], but might be related to the fact that these patients were already on LA supplementation, being a regular treatment of those patients for at least a decade [ 89 , 90 ]. The results might, thus, raise the question if the patients really have a salt deficiency, when the ENaC in both airways and kidneys are hyperactive.…”

“…Nevertheless, the LA supplementation as a routine has not increased inflammation and elastase, and TGF-α have not been found to increase in the patients (Wretlind and Strandvik, unpublished observation). This routine supplementation used for decades must be considered safe [ 89 , 90 ].…”