Abstract

SummaryIn 2 children with glucose‐galactose malabsorption the intermediate metabolism of hexoses was studied. The removal rate of injected glucose was slow when the diet of the children contained limited amounts of well absorbed carbohydrate (fructose). When sufficient fructose was given in the diet, the half‐life of injected glucose was normal. A fast removal rate was seen when large amounts of carbohydrates had been administered prior to the intravenous GTT. The results of oral fructose tolerance tests varied with the pre‐test diet in a similar manner.The fructosemia was small and independent of the diet, but the blood glucose concentration reacted in a way indicating decreased glucose tolerance if the diet contained little absorbable carbohydrate. This was evidently an unspecific effect of carbohydrate deprivation; in a control case, an infant with cystic fibrosis, initially malnourished, signs of decreased glucose tolerance were seen with an oral fructose tolerance test. In him, as well as in the patient with glucose‐galactose malabsorption, the supranorma1 rise of blood glucose was abolished by dietary measures ensuring an ample supply of calories and carbohydrates.The intravenous galactose tolerance, tested in one of the patients, was probably normal at 13 months of age and certainly normal at 6 years of age.It is concluded that the observed abnormalities of the metabolism of glucose in patients with glucose‐galactose malabsorption are unspecific for this disease and merely secondary to carbohydrate deprivation.

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