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一名间歇性低血糖儿童的甘油不耐受情况。

Glycerol intolerance in a child with intermittent hypoglycemia.

作者信息

Maclaren N K, Cowles C, Ozand P T, Shuttee R, Cornblath M

出版信息

J Pediatr. 1975 Jan;86(1):43-9. doi: 10.1016/s0022-3476(75)80702-5.

DOI:10.1016/s0022-3476(75)80702-5
PMID:162954
Abstract

A 3-yr-old boy was investigated for numerous episodes of fatigue, irritability, pallor, and sweating, which began at 11 mo of age, when he had an episode of symptomatic hypoglycemia with ketonuria. He had euphoria, mental confusion, drowsiness, nausea, and vomiting 1-5 hr after oral administration of glycerol in doses of 0.5-1.0gm/kg. Orally administered MCT (1 gm/kg) had similar effects. On one occasion, oral glycerol also provoked hypoglycemia, as had a 16 1/2 hr fast. Intravenously administered glycerol (0.09 gm/kg) induced an immediate loss of consciousness from which he recovered spontaneously after 30 min; there were no changes in blood glucose values. Intravenously administered fructose (0.25 gm/kg) was tolerated normally. Leukocytes showed normal activities for FDPase, glycerol kinase, and glycerol phosphate dehydrogenase. The restriction of dietary intake of fat has been associated with a marked improvement in physical and mental activities. These observations suggest a unique, yet undifined intolerance to glycerol, which suggest caution in the diagnostic use of glycerol in the investigation of hypoglycemia as well as in the therapy of increased intracranial or intraocular pressure.

摘要

一名3岁男孩因多次出现疲劳、易怒、面色苍白和出汗症状接受检查,这些症状始于11个月大时,当时他出现了一次伴有酮尿症的症状性低血糖。口服0.5 - 1.0克/千克剂量的甘油后1 - 5小时,他出现了欣快感、精神错乱、嗜睡、恶心和呕吐。口服中链甘油三酯(1克/千克)也有类似效果。有一次,口服甘油也引发了低血糖,禁食16个半小时也出现了同样情况。静脉注射甘油(0.09克/千克)导致立即失去意识,30分钟后自行恢复;血糖值无变化。静脉注射果糖(0.25克/千克)耐受性正常。白细胞的果糖二磷酸酶、甘油激酶和甘油磷酸脱氢酶活性正常。限制脂肪的饮食摄入与身心活动的显著改善有关。这些观察结果表明对甘油存在一种独特但尚未明确的不耐受性,这提示在低血糖调查以及颅内或眼压升高的治疗中,使用甘油进行诊断时需谨慎。

相似文献

1
Glycerol intolerance in a child with intermittent hypoglycemia.一名间歇性低血糖儿童的甘油不耐受情况。
J Pediatr. 1975 Jan;86(1):43-9. doi: 10.1016/s0022-3476(75)80702-5.
2
Glucoregulation during exercise: hypoglycemia is prevented by redundant glucoregulatory systems, sympathochromaffin activation, and changes in islet hormone secretion.运动期间的血糖调节:通过冗余的血糖调节系统、交感嗜铬细胞激活以及胰岛激素分泌的变化来预防低血糖。
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Response to 2-deoxy-D-glucose and to glucagon in "ketotic hypoglycemia" of childhood: evidence for epinephrine deficiency and altered alanine availability.
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Metabolic and hormonal effects of ethanol.乙醇的代谢和激素效应。
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Glycerol-induced hypoglycemia: a syndrome associated with multiple liver enzyme deficiencies. Clinical and in vitro studies.甘油诱导的低血糖症:一种与多种肝酶缺乏相关的综合征。临床及体外研究。
Metabolism. 1982 Oct;31(10):1057-64. doi: 10.1016/0026-0495(82)90152-4.
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Hepatic fructose-1,6-diphosphatase deficiency. A cause of lactic acidosis and hypoglycemia in infancy.肝果糖-1,6-二磷酸酶缺乏症。婴儿期乳酸酸中毒和低血糖的一个病因。
J Clin Invest. 1972 Aug;51(8):2115-23. doi: 10.1172/JCI107018.
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Attenuated glucose recovery from hypoglycemia in the elderly.
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Hypoinsulinemia is not critical to glucose recovery from hypoglycemia in humans.低胰岛素血症对人体从低血糖中恢复血糖并不关键。
Am J Physiol. 1991 Jul;261(1 Pt 1):E41-8. doi: 10.1152/ajpendo.1991.261.1.E41.
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The rapid changes of hepatic glycolytic enzymes and fructose-1,6-diphosphatase activities after intravenous glucagon in humans.静脉注射胰高血糖素后人体肝脏糖酵解酶和果糖-1,6-二磷酸酶活性的快速变化。
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Glucagon, not insulin, may play a secondary role in defense against hypoglycemia during exercise.在运动期间抵御低血糖的过程中,发挥次要作用的可能是胰高血糖素,而非胰岛素。
Am J Physiol. 1988 Jun;254(6 Pt 1):E713-9. doi: 10.1152/ajpendo.1988.254.6.E713.

引用本文的文献

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Glycerol intoxication syndrome in young children, following the consumption of slush ice drinks.幼儿饮用碎冰饮料后发生甘油中毒综合征。
Arch Dis Child. 2025 Jul 18;110(8):592-596. doi: 10.1136/archdischild-2024-328109.
2
Issues in the formulation of drugs for oral use in children: role of excipients.儿童口服制剂的配方问题:辅料的作用
Paediatr Drugs. 2002;4(6):371-9. doi: 10.2165/00128072-200204060-00004.
3
Pharmaceutical excipients. Adverse effects associated with 'inactive' ingredients in drug products (Part II).
药用辅料。药品中与“非活性”成分相关的不良反应(第二部分)。
Med Toxicol Adverse Drug Exp. 1988 May-Jun;3(3):209-40. doi: 10.1007/BF03259883.
4
Fructose-1,6-diphosphatase deficiency: glycerol excretion during fasting test.果糖-1,6-二磷酸酶缺乏症:禁食试验期间的甘油排泄
Eur J Pediatr. 1985 Jul;144(2):203-4. doi: 10.1007/BF00451915.