Abstract
ABSTRACT. Fernandes, J. (Department of Paediatrics, Sophia Children's Hospital and Neonatal Unit, Erasmus University, Rotterdam, The Netherlands). The effect of disaccharides on the hyperlactacidaemia of glucose‐6‐phosphatase‐deficient children. Acta Paediat Scand,63:695, 1974.—Two unrelated children with hepatic glycogenosis due to glucose‐6‐phosphatase deficiency were investigated as regards the influence of dietary disaccharides on their hyperlactacidaemia. The disaccharides were administered orally as 10% solutions hourly during 12 hours in an A‐B‐A sequence, i.e. maltose‐lactose‐maltose, or maltose‐sucrose‐maltose. Each disaccharide was given in repeated doses. Blood lactate levels increased during the lactose or sucrose feedings and decreased during the maltose feedings, while normoglycaemia was maintained throughout the experiments. It is concluded that lactose and sucrose should be restricted in the diet of children with a glucose‐6‐phosphatase deficiency.
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