Familial Hypoglycemia Precipitated by Amino Acids

Abstract

This paper reports three cases of hypoglycemia in one family and an unrelated case in which profound hypoglycemia and convulsions were produced by the administration of proteins or amino acids. The patients described are similar to the group which have been reported in the literature as infantile idiopathic hypoglycemosis. Casein, leucine, and isovaleric acid fed to the patients caused a marked fall in the fasting concentration of true blood sugar. High protein feeding led to more convulsions and lower concentrations of sugar in the blood than did a low protein diet. Leucine had the most dramatic effect. Casein and leucine did not produce a fall in the sugar in the blood in normal individuals. The literature on metabolic interrelations between amino acids and carbohydrates and the concentration of sugar in the blood is reviewed. The mode of action of amino acids in depressing the concentration of sugar in the blood in infants with spontaneous hypoglycemia is considered and several hypotheses proposed. This paper is of unusual interest and of great importance for the treatment of infants with spontaneous idiopathic hypoglycemia. It is suggested that in this particular type of hypoglycemia high protein diets should be avoided. Small amounts of carbohydrate may be given 30 to 40 minutes after the ingestion of a meal containing protein to offset the effect of protein in lowering the concentration of sugar in the blood.