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Familial Hypoglycemia Precipitated by Amino Acids

机译:氨基酸引起的家族性低血糖症

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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.
机译:本文报道了一个家庭中的三例低血糖症,与一个无关的病例,其中通过蛋白质或氨基酸的给药产生了严重的低血糖症和惊厥。所描述的患者与文献中报道的婴儿特发性低血糖症相似。喂给患者的酪蛋白,亮氨酸和异戊酸会导致空腹血糖下降。与低蛋白饮食相比,高蛋白喂养导致更多的惊厥和血液中糖分的降低。亮氨酸的作用最为显着。在正常个体中,酪蛋白和亮氨酸不会使血液中的糖分下降。氨基酸和碳水化合物之间的代谢相互关系和血液中糖的浓度的文献进行了审查。考虑了氨基酸降低自发性低血糖婴儿血液中糖浓度的作用方式,并提出了几种假设。本文对治疗自发性特发性低血糖症的婴儿具有非同寻常的兴趣,并且具有重要意义。建议在这种特殊的低血糖类型中,应避免高蛋白饮食。进食含蛋白质的餐后30至40分钟可给予少量碳水化合物,以抵消蛋白质降低血液中糖浓度的作用。

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