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Main urinary bacterial metabolites of phenylalanine (total benzoic and phenylacetic acids) and of tyrosine (total p-hydroxybenzoic acid and p-hydroxyphenylacetic acid) were determined by gas chromatography in controls and patients with cystic ubrosis of the pancreas, coeliac disease, intestinal resection and unclassified enteritis. In various patients, especially in the untreated coeliacs, high amounts of one or more of the abovementioned metabolites were found. In this paper results in controls and patients are presented and discussed.
Tyrosyluria and for a part also tyrosinemia were studied in 60 healthy prematures of various birth weights and gestational ages. The first analyses were performed between the 6th and the 14th day after birth. A normal milk diet was given and the protein-intake was between 3 and 4 g/kg. After the first collection of urine half the patients received extra ascorbic acid, 100 mg/kg daily. Urinary analyses of tyrosine and p-hydroxyphenyl metabolites were performed once a week, until the excretion of p-hydroxyphenylpyruvic plus p-hydroxyphenyllactic acids was lower than 5 mmoles per gram creatinine. In 22 out of the 60 prematures (or 37%) a tyrosyluria of more than 5 mmoles/g creatinine and in 19 out of 44 (43%) patients analysed serum tyrosine was higher than 5 mg/100 ml at first analysis. No inverse correlation between tyrosyluria and t...
Gas-liquid chromatographic methods have been developed for the analysis of: urinary phenylalanine metabolites (I) in patients with phenylketonuria, tyrosine metabolites (II) in patients with a disturbed tyrosine metabolism at the level of p-hydroxyphenylpyruvate hydroxylase, and homogentisic acid in alkaptonuria. Metabolites I include: phenylpyruvic, -lactic, -acetic (free and conjugated), -mandelic, o-hydroxyphenylacetic and benzoic (free and conjugated) acids. Metabolites II include: p-hydroxyphenylpyruvic, -lactic, -acetic and p-hydroxymandelic acids. Urinary excretions of phenylalanine and its waste metabolites I in patients with phenylalanine hydroxylase deficiency, at high and moderate loads are given. In 3 patients with classical phenylketonuria the total excretion of phenylalanine and its waste metabolites were found to b...
Chromatographic characteristics of urinary metabolites of theophylline were studied by two-dimensional thin-layer chromatography, high-performance liquid chromatography and gas chromatography—mass spectrometry. Quantitative data for the urinary metabolites of theophylline in asthmatic children are given. It was shown that 1,3-dimethyluric acid is the predominant excretory product. In addition, smaller amounts of 1-methyluric acid, 3-methylxanthine and unchanged theophylline were found. Excretory patterns after theophylline ingestion before and during the administration of allopurinol in asthma patients and in rats suggest the existence of three metabolic pathways of theophylline. The administration of this drug to a patient with xanthine oxidase deficiency resulted in the excretion of 1-methyluric acid in addition to 1,3-dimethyluric ...
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