HYPOALANINEMIA - CONCOMITANT OF KETOTIC HYPOGLYCEMIA
HYPOALANINEMIA - CONCOMITANT OF KETOTIC HYPOGLYCEMIA
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DOI：
10.1172/jci106940
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发表时间：
1972-01-01
影响因子：
15.9
通讯作者：
FEIGIN, RD
中科院分区：
文献类型：
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作者：
PAGLIARA, AS;KIPNIS, DM;FEIGIN, RD
酮症低血糖是儿童期最常见的低血糖，其病因尚不清楚。本研究旨在确定这种情况的主要缺陷是肝致坏死前体缺乏还是肝致坏死酶系统异常。在8名酮症低血糖儿童和7名年龄匹配的对照组中测定了血糖、丙氨酸、胰岛素和血β-羟基丁酸（β-OHB）、丙酮酸和乳酸水平，这些儿童维持正常饮食，并在喂食刺激性低热量低碳水化合物饮食（1200 kcal/1.73 m2，15%碳水化合物，17%蛋白质和68%脂肪）后进行了测定。正常饮食，空腹过夜血浆丙氨酸（211±10 μM）和葡萄糖（68±4 mg/100 ml）显著降低，血β-OHB（1.22±0.37 mM）显著高于对照组（丙氨酸，315±15 μM;葡萄糖，81±3 mg/100 ml; β-OHB，0.18±0.08 mM）。所有酮症低血糖儿童均发生症状性低血糖（33±3 mg/100 ml）和酮症（β-OHB，3.70±0.32 mM），这些变化与血浆丙氨酸（155±17 μM）的进一步下降相关。正常儿童在此饮食36小时后仍维持较高的血糖（48±2 mg/100 ml）和丙氨酸（225±5 μM），较低的β-OHB水平（2.56±0.44 mM）。静脉输注丙氨酸（250 mg/kg）可使酮症低血糖儿童的低血糖血糖水平恢复正常。醋酸可的松（300 mg/m2），在喂食刺激性饮食期间分三次口服给药，在开始类固醇治疗后4-6小时内使血浆丙氨酸增加3- 4倍，并完全防止了低血糖和酮症的发生。进行定量氨基酸谱，并证明丙氨酸是两组之间显著不同的唯一致炎氨基酸。在所有检查条件下，正常和酮症低血糖儿童的血浆胰岛素和血乳酸及丙酮酸水平没有显著差异。这些结果支持了以下假设：丙氨酸）而不是肝促血管生成酶装置的缺陷代表了酮性低血糖发病机制中最可能的因素。
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).All ketotic hypoglycemic children developed symptomatic hypoglycemia (33±3 mg/100 ml) and ketosis (β-OHB, 3.70±0.32 mM) 8-16 hr after starting the provocative diet and these changes were associated with a further decline in plasma alanine (155±17 μM). Normal children, even after 36 hr on this diet, maintained higher plasma glucose (48±2 mg/100 ml) and alanine (225±5 μM) and lower β-OHB levels (2.56±0.44 mM).Intravenous infusions of alanine (250 mg/kg) uniformly restored the hypoglycemic plasma glucose levels of ketotic hypoglycemic children to normal. Cortisone acetate (300 mg/m2), given orally in three divided doses during feeding of the provocative diet, produced a 3- to 4-fold increase in plasma alanine within 4-6 hr after beginning steroid therapy and completely prevented the development of hypoglycemia and ketosis. Quantitative amino acid profiles were performed and demonstrated that alanine was the only gluconeogenic amino acid which differed significantly between the two groups. Plasma insulin and blood lactate and pyruvate levels did not differ significantly between normal and ketotic hypoglycemic children under all conditions examined.These results support the hypothesis that a deficiency in gluconeogenic precursor (e.g., alanine) rather than a defect in the hepatic gluconeogenic enzyme apparatus represents the most likely factor in the pathogenesis of ketotic hypoglycemia.