摘要: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.