Organic aciduria in hypoxic premature newborns simulating an inborn error of metabolism
✍ Scribed by J. A. J. M. Bakkeren; R. C. A. Sengers; J. M. F. Trijbels; P. H. A. Th. Engels
- Publisher
- Springer
- Year
- 1977
- Tongue
- English
- Weight
- 376 KB
- Volume
- 127
- Category
- Article
- ISSN
- 0340-6997
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✦ Synopsis
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, a-hydroxy-butyric acid, fl-hydroxy-butyric acid, a-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.