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Ketosis in Hepatic Glycogenosis

The occurrence of ketosis in 41 patients with liver glycogenosis and a control group of 22 children was investigated. Fasting ketosis was present in children with a deficiency of the debranching enzyme system and in young children with a deficiency of the phosphorylase system, but never in patients with a glucose-6-phosphatase deficiency. Oral tolerance tests on a patient deficient in debranching enzyme showed that the blood levels of glucose and ketone bodies changed in the opposite direction. It is argued that, for biochemical reasons, the occurrence of ketosis in patients with a glucose-6-phosphatase deficiency is improbable.