Plasma lipid concentrations in children with cystic fibrosis: the value of a high-fat diet and pancreatic supplementation.

Impaired digestion of dietary fat is an almost universal feature of cystic fibrosis (CF) which results in low concentrations of essential fatty acids in plasma lipids. We have evaluated the effect of a high-lipid diet and pancreatic enzyme supplementation, using enteric-coated microsphere preparations, on plasma lipid concentrations in paediatric CF patients. Absorption of dietary lipid was comparable between control and CF subjects. This resulted in plasma cholesterol, triacylglycerol, total phosphatidylcholine and individual phosphatidylcholine molecular species concentrations in CF patients which were in the same range as those in controls. Normal values for these variables were also found in patients with clinically detectable liver disease. These results show that present dietary management of CF patients supports normal plasma lipid concentrations.