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缺氧早产新生儿的有机酸尿症模拟先天性代谢缺陷。

Organic aciduria in hypoxic premature newborns simulating an inborn error of metabolism.

作者信息

Bakkeren J A, Sengers R C, Trijbels J M, Engels P H

出版信息

Eur J Pediatr. 1977 Dec 30;127(1):41-7. doi: 10.1007/BF00465564.

DOI:10.1007/BF00465564
PMID:606558
Abstract

A very unusual pattern of organic acid excretion was established in the urine of two premature newborns with severe respiratory distress and cerebral haemorrhages. By combined gas chromatography/mass spectrometry the following acids were identified: lactic acid, alpha-hydroxy-butyric acid, beta-hydroxy-butyric acid, alpha-hydroxy-isovaleric acid, and p-hydroxy-phenyllactic acid. Calculation of the concentration revealed an excessive excretion of lactic acid and also very high excretion of the other acids. A postmortem blood sample from one of the patients revealed a comparable pattern. The abnormal urinary organic acid excretion pattern was most probably caused by severe tissue hypoxia.

摘要

在两名患有严重呼吸窘迫和脑出血的早产新生儿尿液中,发现了一种非常不寻常的有机酸排泄模式。通过气相色谱/质谱联用技术,鉴定出了以下几种酸:乳酸、α-羟基丁酸、β-羟基丁酸、α-羟基异戊酸和对羟基苯乳酸。浓度计算显示,乳酸排泄过量,其他酸的排泄量也非常高。其中一名患者的尸检血样显示出类似的模式。异常的尿有机酸排泄模式很可能是由严重的组织缺氧引起的。

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2
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本文引用的文献

1
Increased urinary excretion of 3-hydroxyisovaleric acid in patients with ketoacidosis.
Clin Chim Acta. 1974 Jul 15;54(1):39-46. doi: 10.1016/0009-8981(74)90040-0.
2
[Comparative study of urine and serum organic acids in newborn infants using gas chromatography].[采用气相色谱法对新生儿尿液和血清有机酸的比较研究]
Union Med Can. 1974 Apr;103(4):678-85.
3
Amino acid excretion of premature infants receiving different amounts of protein.接受不同蛋白量的早产儿的氨基酸排泄情况。
Clin Chim Acta. 1973 Nov 23;49(1):27-39. doi: 10.1016/0009-8981(73)90339-2.
年龄相关诊断(ARD)指数简介:一种用于诊断的就诊年龄相关指数。
J Inherit Metab Dis. 1993;16(2):161-70. doi: 10.1007/BF00710248.
4
The effects of fetal energy depletion on amniotic fluid concentrations of amino acids, organic acids and related metabolites.胎儿能量消耗对羊水中氨基酸、有机酸及相关代谢物浓度的影响。
J Inherit Metab Dis. 1988;11(1):103-13. doi: 10.1007/BF01800060.
4
A micro-method for the enzymatic determination of acetoacetate and 3-hydroxybutyrate in blood and urine.一种用于酶法测定血液和尿液中乙酰乙酸和3-羟基丁酸的微量方法。
Scand J Clin Lab Invest. 1970 Mar;25(2):171-9. doi: 10.3109/00365517009049200.
5
Beta-hydroxyisovaleric aciduria and beta-methylcrotonylglycinuria: a new inborn error of metabolism.β-羟基异戊酸尿症和β-甲基巴豆酰甘氨酸尿症:一种新的先天性代谢缺陷。
Lancet. 1970 Sep 5;2(7671):521-2. doi: 10.1016/s0140-6736(70)90140-6.
6
Beta-methylcrotonic aciduria associated with lactic acidosis.与乳酸性酸中毒相关的β-甲基巴豆酸尿症。
J Pediatr. 1976 Feb;88(2):229-35. doi: 10.1016/s0022-3476(76)80987-0.
7
Organic acids in urine from human newborns.
Clin Chem. 1976 Jan;22(1):49-52.
8
Clinical conditions associated with urinary excretion of 2-hydroxybutyric acid.与2-羟基丁酸尿排泄相关的临床病症。
Scand J Clin Lab Invest. 1975 May;35(3):259-66.