Value of the glucagon test in screening for hepatic glycogen storage disease.

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Abstract
The fasting glucagon test of 40 patients with hepatic glycogen storage disease (type I, 13 patients; type Ib, 5 patients; type III, 12 patients; type IX (phosphorylase kinase deficiency), 10 patients) was reviewed. In all patients with types Ib and III, the blood glucose level rose < 1 mmol/l, but in types I and IX there was wide variation in response. Blood lactate levels were > 2.4 mmol/l in all patients with type I and Ib 120 min after the administration of glucagon but were < 2.4 mmol/l in types III and IX. The value of this test for screening was analyzed using several criteria. A simplified scheme for the investigation of patients with suspected hepatic glycogen storage disease is proposed.