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儿童酮症性低血糖——几种统一病因假说的临床试验

Ketotic hypoglycemia of childhood--a clinical trial of several unifying etiological hypotheses.

作者信息

Dahlquist G, Gentz J, Hagenfeldt L, Larsson A, Löw H, Persson B, Zetterström R

出版信息

Acta Paediatr Scand. 1979 Sep;68(5):649-56. doi: 10.1111/j.1651-2227.1979.tb18433.x.

DOI:10.1111/j.1651-2227.1979.tb18433.x
PMID:393061
Abstract

We have studied 15 children referred to St. Göran's Children's Hospital because of suspected ketotic hypoglycemia. The patients were investigated according to a program designed to test several hypotheses--old and new--postulated to explain the etiology of ketotic hypoglycemia. We have used the classical ketogenic provocation with a low calorie, high fat diet and measured the blood levels of several substrates and hormones as well as the urinary excretion of certain metabolites and hormones. Out of the 15 children, 6 will fill the criteria of ketotic hypoglycemia at the time of study. The most remarkable finding in these 6 children in contrast to the other children studied was that they did not decrease their peripheral glucose utilization (measured as Kg) during starvation. These 6 children seemed to be more "advanced" in their adaptation to ketogenic diet in all other parameters studied. The children with ketotic hypoglycemia did not differ from the other children in plasma level of cortisol or urinary excretion of nitrogen, urea, 3-methylstidine and catecholamines. We favour the concept that the children with ketotic hypoglycemia represent the tail of the gaussian curve in the normal age-dependent development of the adaptation to starvation.

摘要

我们研究了15名因疑似酮症性低血糖症而转诊至圣戈兰儿童医院的儿童。根据一个旨在检验若干用以解释酮症性低血糖症病因的新旧假说的方案,对这些患者进行了调查。我们采用了低热量、高脂肪饮食的经典生酮激发试验,并测量了几种底物和激素的血药浓度以及某些代谢物和激素的尿排泄量。在这15名儿童中,有6名在研究时符合酮症性低血糖症的标准。与其他接受研究的儿童相比,这6名儿童最显著的发现是,他们在饥饿期间并未降低外周葡萄糖利用率(以Kg衡量)。在所有其他研究参数方面,这6名儿童在适应生酮饮食方面似乎更为“先进”。酮症性低血糖症患儿与其他儿童在皮质醇血浆水平或氮、尿素、3-甲基组氨酸和儿茶酚胺的尿排泄量方面并无差异。我们支持这样一种观点,即酮症性低血糖症患儿代表了正常年龄依赖性饥饿适应发育过程中高斯曲线的尾部。

相似文献

1
Ketotic hypoglycemia of childhood--a clinical trial of several unifying etiological hypotheses.儿童酮症性低血糖——几种统一病因假说的临床试验
Acta Paediatr Scand. 1979 Sep;68(5):649-56. doi: 10.1111/j.1651-2227.1979.tb18433.x.
2
Hypoalaninemia: a concomitant of ketotic hypoglycemia.低丙氨酸血症:酮症性低血糖的一个伴随症状。
J Clin Invest. 1972 Jun;51(6):1440-9. doi: 10.1172/JCI106940.
3
Glycemic response to 24 hour fast in normal children and children with ketotic hypoglycemia.正常儿童和酮症性低血糖儿童对24小时禁食的血糖反应。
J Pediatr. 1973 Mar;82(3):438-43. doi: 10.1016/s0022-3476(73)80117-9.
4
[Significance of the 2-deoxy-d-glucose-test on the differentiation of hypoglycemia in childhood (author's transl)].
Klin Padiatr. 1975 Jul;187(4):308-13.
5
Hypoketosis as a cause of symptoms in childhood hypoglycemia.
Eur J Pediatr. 1980 Jun;134(1):51-5. doi: 10.1007/BF00442403.
6
[Hypoglycemia in children].[儿童低血糖症]
Rev Med Brux. 1997 Oct;18(5):339-46.
7
Ketotic hypoglycemia and the ketogenic diet.酮症性低血糖与生酮饮食。
Neurology. 1973 Jun;23(6):640-9. doi: 10.1212/wnl.23.6.640.
8
Studies of children with ketotic hypoglycemia.对酮症性低血糖儿童的研究。
Yale J Biol Med. 1970 Jun;42(6):420-38.
9
Fasting ketone levels vary by age: implications for differentiating physiologic from pathologic ketotic hypoglycemia.空腹酮体水平因年龄而异:对区分生理性与病理性酮症性低血糖的意义。
J Pediatr Endocrinol Metab. 2023 May 11;36(7):667-673. doi: 10.1515/jpem-2022-0589. Print 2023 Jul 26.
10
Ketotic hypoglycemia in a four-year-old boy with adrenal cortical insufficiency.
Acta Paediatr Scand. 1972 Jul;61(4):465-9. doi: 10.1111/j.1651-2227.1972.tb15865.x.

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Horm Res Paediatr. 2024 Mar 21:1-12. doi: 10.1159/000538483.
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PHKA2 variants expand the phenotype of phosphorylase B kinase deficiency to include patients with ketotic hypoglycemia only.PHKA2 变异扩大了磷酸化酶激酶缺乏症的表型,包括仅出现酮性低血糖的患者。
Am J Med Genet A. 2021 Oct;185(10):2959-2975. doi: 10.1002/ajmg.a.62383. Epub 2021 Jun 12.
3
Towards enhanced understanding of idiopathic ketotic hypoglycemia: a literature review and introduction of the patient organization, Ketotic Hypoglycemia International.
为了增进对特发性酮症低血糖症的理解:文献回顾及患者组织酮症低血糖国际的介绍。
Orphanet J Rare Dis. 2021 Apr 13;16(1):173. doi: 10.1186/s13023-021-01797-2.
4
Fasting adaptation in idiopathic ketotic hypoglycemia: a mismatch between glucose production and demand.特发性酮症低血糖中的禁食适应:葡萄糖生成与需求之间的不匹配。
Eur J Pediatr. 2008 Aug;167(8):859-65. doi: 10.1007/s00431-007-0598-5. Epub 2007 Oct 13.
5
From hyperinsulinaemic hypoglycaemia to ketotic hypoglycaemia: the range of glucose abnormalities in patients born with intrauterine growth retardation.从高胰岛素血症性低血糖到酮症性低血糖:宫内生长受限患儿的血糖异常范围
Eur J Pediatr. 2007 Oct;166(10):1003-7. doi: 10.1007/s00431-006-0371-1. Epub 2006 Dec 20.
6
Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia.特发性酮症性低血糖症中的葡萄糖和亮氨酸动力学
Arch Dis Child. 2006 Jun;91(6):483-6. doi: 10.1136/adc.2005.089425. Epub 2006 Jan 27.
7
Ketotic hypoglycaemia in children with diazoxide responsive hyperinsulinism of infancy.患有对二氮嗪有反应的婴儿期高胰岛素血症的儿童的酮症性低血糖症。
Eur J Pediatr. 2005 Jun;164(6):387-90. doi: 10.1007/s00431-005-1654-7. Epub 2005 Mar 17.
8
Hypoalaninemia and ketotic hypoglycemia: cause or consequence?低丙氨酸血症和酮症性低血糖:是原因还是结果?
Eur J Pediatr. 1982 Feb;138(1):28-31. doi: 10.1007/BF00442324.