ORGANIC ACIDURIA IN HYPOXIC PREMATURE NEWBORNS SIMULATING AN INBORN ERROR OF METABOLISM
ORGANIC ACIDURIA IN HYPOXIC PREMATURE NEWBORNS SIMULATING AN INBORN ERROR OF METABOLISM
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DOI：
10.1007/bf00465564
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发表时间：
1977-01-01
影响因子：
3.6
通讯作者：
ENGELS, PHAT
中科院分区：
文献类型：
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作者：
BAKKEREN, JAJM;SENGERS, RCA;ENGELS, PHAT
在2名患有严重呼吸窘迫和脑出血的早产儿的尿液中发现了一种非常不寻常的有机酸排泄模式。通过组合的气相色谱法/质谱法，鉴定出以下酸：乳酸，α-羟基丁酸，β-羟基丁酸，α-羟基-异戊酸和对羟基-苯基乳酸。浓度计算显示乳酸过量排泄，其他酸的排泄也非常高。其中一名患者的尸检血样显示了类似的模式。尿有机酸排泄模式异常很可能是由严重的组织缺氧引起的。
A very unusual pattern of organic acid excretion was established in the urine of 2 premature newborns with severe respiratory distress and cerebral hemorrhages. 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 post mortem 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.