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 cy stic fibrosis (CF) which results in low concentrations of essential fa tty acids in plasma lipids. We have evaluated the effect of a high-lip id diet and pancreatic enzyme supplementation, using enteric-coated mi crosphere preparations, on plasma lipid concentrations in paediatric C F patients. Absorption of dietary lipid was comparable between control and CF subjects. This resulted in plasma cholesterol, triacylglycerol , total phosphatidylcholine and individual phosphatidylcholine molecul ar species concentrations in CF patients which were in the same range as those in controls. Normal values for these variables were also foun d in patients with clinically detectable liver disease. These results show that present dietary management of CF patients supports normal pl asma lipid concentrations.