STUDIES OF GLYCOGEN METABOLISM IN LIVER GLYCOGEN DISEASE (VON GIERKE'S DISEASE): SIX CASES WITH SIMILAR METABOLIC ABNORMALITIES AND RESPONSES TO GLUCAGON*

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Abstract
Six children with liver glycogen disease had strikingly elevated fasting blood lactate levels, averaging 85 mg%. In all 5 so studied, the blood lactate concentration fell when hypoglycemia was corrected by feeding or glucose infusions and rose again after hypoglycemia was allowed to recur. In all 6 children, glucagon injection resulted in little or no rise in blood glucose but in prompt and large increase of blood lactate levels. Intravenous infusions of glucagon produced acute decreases of liver size. More lactate than glucose was produced during in vitro incubation of liver slices. Stimulation of glycogenolysis by glucagon during such incubation was demonstrated in one case, but only traces of glucose appeared in the medium. It is concluded that active breakdown of liver glycogen occurred in these patients in response to hypoglycemia and that glucagon exerted its usual hepatic glycogenolytic effect. However, the principal end-product of liver glycogen breakdown, whether spontaneous or glucagon-induced, was lactic acid rather than glucose.