STUDIES ON DIETARY CORRECTION OF METABOLIC ABNORMALITIES IN HEPATORENAL GLYCOGENOSIS

Extract: This study reports the effects of frequent glucose feeding on the metabolic abnormalities observed in a patient with hepatorenal glycogenosis. Use of this diet resulted in correction of metabolic acidosis and hyperlacticacidemia. External calcium balance became positive secondary to a decrease in the loss of calcium in urine. Long-term use of the diet caused a reduction to normal values of triglyceride and cholesterol levels in serum. Continued use of the diet for nine months resulted in increased growth rate and increased calcification of bone. Evidence based on excretion of epinephrine and 17-hydroxy-corticosteroids in urine did not indicate that an increased excretion of these hormones occurred in response to hypoglycemia. Concentrations of growth hormone were low despite hypoglycemia; however, after nine months of treatment, the patient showed a rapid decline in the levels of blood sugar with an increase in the levels of growth hormone in blood. Speculation:Partially controlled hepatorenal glycogenosis is in some ways similar to juvenile diabetes mellitus since patients with this disease sustain intermittent starvation of the peripheral tissues for glucose. Study of the metabolic sequellae of peripheral glucose deprivation and repletion in some forms of glycogenosis may lead to a better understanding of the complications of juvenile diabetes mellitus. © International Pediatrics Research Foundation, Inc. 1969. All Rights Reserved.