Hypoalaninemia: a concomitant of ketotic hypoglycemia

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Abstract

The cause of of ketotic hypoglycemia, the commonest form of hypoglycemia in childhood, is not known. The present study was undertaken to determine whether the primary defect in this condition is a deficiency of gluconeogenic precursor(s) or an abnormality in the hepatic gluconeogenic enzyme system. Plasma glucose, alanine, and insulin and blood β-hydroxybutyrate (β-OHB), pyruvate, and lactate levels were determined in eight ketotic hypoglycemic children and seven agematched controls maintained on a normal diet and after being fed a provocative hypocaloric low-carbohydrate diet (1200 kcal/1.73 m2, 15% carbohydrate, 17% protein, and 68% fat). On a normal diet, overnight fasting plasma alanine (211±10 μM) and glucose (68±4 mg/100 ml) were significantly lower and blood β-OHB (1.22±0.37 mM) significantly higher in ketotic hypoglycemic children than in controls (alanine, 315±15 μM; glucose, 81±3 mg/100 ml; β-OHB, 0.18±0.08 mM).

Authors

Anthony S. Pagliara, Irene E. Karl, Darryl C. De Vivo, Ralph D. Feigin, David M. Kipnis