Examination of urine metabolites in the newborn period and during protein loading tests at 6 months of age--Part 1

European Journal of Pediatrics
E MönchU Batzler

Abstract

In the course of the collaborative study of children treated for PKU, urine samples from a total of 165 patients were analysed at six different times: in the newborn period before onset of therapy, after beginning of dietary management, during and immediately after a protein loading test at 6 months of age. In 95.9% of newborns with elevated Phe levels in plasma, metabolites of this amino acid as well as of Tyr could be detected. Of all metabolites phenylpyruvate always showed the highest concentration, followed by phenyllactate and o-hydroxy-phenylacetate. During the protein loading test an increase of the same metabolites occurred. At the age of 6 months the percentage of p-hydroxylated compounds related to the sum of all metabolites was lower than in the newborn period. Comparing the results of urine analyses at 6 months of age after the protein loading tests with the classification of HPA into the reaction types I-III, it can be clearly stated that patients with the milder forms II and III have already lower levels of Phe metabolites in urine before onset of therapy compared to the reaction type I. In retrospect 52% of the newborns could therefore be classified as reaction type I even before beginning of dietary management....Continue Reading

References

Sep 30, 1974·Clinica Chimica Acta; International Journal of Clinical Chemistry·R A Chalmers, R W Watts
Nov 1, 1974·Archives of Disease in Childhood·M E BlaskovicsS Hack
May 18, 1973·Clinica Chimica Acta; International Journal of Clinical Chemistry·K BlauC H Edwards
Sep 14, 1973·Clinica Chimica Acta; International Journal of Clinical Chemistry·H J SternowskyG Gaull
Mar 1, 1972·Clinica Chimica Acta; International Journal of Clinical Chemistry·A HillW A Zaleski
Oct 1, 1969·Analytical Biochemistry·N E Hoffman, K M Gooding
Jan 1, 1970·Clinica Chimica Acta; International Journal of Clinical Chemistry·K Blau

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Citations

Jan 1, 1992·Journal of Inherited Metabolic Disease·U LangenbeckA Mench-Hoinowski
Jul 18, 2009·Journal of Inherited Metabolic Disease·U LangenbeckUNKNOWN German Collaborative Study on Phenylketonuria (PKU) / Hyperphenylalaninemia (HPA)
Jan 1, 1990·European Journal of Pediatrics·U WendelU Batzler
Oct 13, 2020·The Science of the Total Environment·Xiaoliang LiaoZongwei Cai

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