PEDIATRICS Vol. 35 No. 3 March 1965, pp. 470-477
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STUDIES ON THE UTILIZATION OF HEXOSES IN LIVER GLYCOGEN DISEASE

J. Fernandes M.D.1 and J. H. van de Kamer Ph.D.2

1 The Children's Department, Protestants Ziekenhuis 's-Hertogenbosch, Utrecht, The Netherlands
2 The Central Institute for Nutrition and Food Research T.N.O., Utrecht, The Netherlands

The utilization of glucose, fructose, and galactose was studied by oral tolerance tests in four patients with liver glycogen disease, two having a phosphorylase deficiency, the other two a glucose-6-phosphatase deficiency.

The utilization of these sugars was evaluated by the estimation of glucose, total reducing capacity, non-esterified fatty acids (NEFA), and lactate in the blood. The data were plotted against time and the curves obtained in the two types of abnormal carbohydrate metabolism and in two control children were compared.

The curves in children with phosphorylase deficiency approached the normal pattern.

In children with glucose-6-phosphatase deficiency, however, the curves for fructose and galactose were highly abnormal.

It was concluded that in the latter case fructose and galactose should be excluded from the diet.

Submitted on April 15, 1964
Accepted on September 29, 1964