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American Journal of Clinical Nutrition, Vol 22, 617-627, Copyright © 1969 by The American Society for Clinical Nutrition, Inc.

Hyperlipemia in Children with Liver Glycogen Disease

J. FERNANDES M.D.1 and N. A. PIKAAR PH.D.1

1 From the Wilhelmina Children's Hospital, University of Utrecht, and the Central Institute for Nutrition and Food Research TNO, Zeist, The Netherlands

Seven children with liver glycogen disease were studied during periods of controlled formula diets. The formula was changed equicalorically by altering either the carbohydrate-fat ratio or the type of fat. Fasting blood samples were taken once a week and analyzed for total fatty acids, cholesterol, phospholipids, and composition of the fatty acids. In addition, the qualitative composition of the lipoproteins was analyzed by paper electrophoresis. The two patients with a glucose-6-phosphatase deficiency were found to have very high serum lipid levels. These were due mainly to hypertriglyceridemia. The percentage of palmitic acid in the fatty acids usually was much above normal, and on paper electrophoretograms a distinct pre-beta band was visible. Lowering the carbohydrate-fat ratio in the diet hardly reduced the hyperlipemia. The patients with deficiency of the debranching enzyme system and with deficiency of the phosphorylase system were less hyperlipemic, while on paper electrophoretograms only a dense beta band was observed but no pre-beta band. Substitution of fat for an equicaloric amount of carbohydrates lowered the lipid level and the palmitic acid percentage, especially in patients with severe hyperlipemia. The nature of the fat in the diet was also important. Corn oil and olive oil were found to have a favorable effect and coconut oil and MCT an unfavorable effect on the hyperlipemia. Substitution, in one experiment, of sucrose for starch had no effect.

These data suggested the mechanisms of causation of the hyperlipemia in the three types of GSD studied. Suitable diets were developed for patients with GSD, types I, III, and VI.




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