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. 1978 Jan 2;82(1-2):93-9.
doi: 10.1016/0009-8981(78)90030-x.

The variability of metabolite excretion in propionicacidaemia

M Duran, D Gompertz, L Bruinvis, D Ketting, S K Wadman

The variability of metabolite excretion in propionicacidaemia

M Duran et al. Clin Chim Acta. .

Abstract

Random urine samples from eight patients with propionicacidaemia were analyzed by gas chromatography and mass spectrometry in order to see if a consistent metabolite pattern with a high diagnostic value could be found. However, wide variations were observed. The presence of 3-hydroxypropionate and/or methylcitrate were considered to be diagnostic of propionyl-CoA carboxylase deficiency. In addition, samples from ketotic periods frequently contained 3-hydroxy-n-valerate and 3-oxo-n-valerate.

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