Response to Medium Chain Triglycerides in von Gierke's Disease

Dietary studies have been carried out on the effects of ingested carbohydrate and fat (as MCT and LCT) on hyperlipemia in a patient with von Gierke's disease. Increased carbohydrate in the diet resulted in increased serum lipid levels represented predominantly in the triglyceride fraction. Substitution of MCT for LCT in the diet when carbohydrate consumption was at normal levels resulted in significantly decreased serum lipid levels, disappearance of eruptive xanthomas, and reduction in liver size. We suggest on the basis of this preliminary study that excess carbohydrate should be avoided in the diet and substitution of MCT for LCT should be considered as a rational approach to management of lipid abnormalities in von Gierke's disease.