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Absence of a significant renal threshold for two aromatic acids in phenylketonuric children over two years of age

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Abstract

There is a steady and nonlinear relationship between the levels of both phenylpyruvic acid (PPA) and o-hydroxyphenylacetic acid (oOPAA) in urine and the plasma levels of phenylalanine (phe) in children more than two years of age with phenylketonuria (PKU). If phe levels in blood rise above 0.35 mM (5.8 mg/100 ml) both aromatic acids are found regularly in urine. Typically, urinary concentrations of PPA are about 5 times higher than those of oOPAA. This report is based on the analysis of 94 samples from 51 children, on or off diet.

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Authors and Affiliations

  1. Institute of Human Genetics, University of Göttingen, Nikolausberger Weg 5a, D-3400, Göttingen, Federal Republic of Germany

    Ulrich Langenbeck, Angelika Mench-Hoinowski & Manfred Petersen

  2. Children's Hospital, University of Göttingen, Humboldtallee 38, D-3400, Göttingen, Federal Republic of Germany

    Abdolwahab Behbehani

Authors
  1. Ulrich Langenbeck
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  2. Abdolwahab Behbehani
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  3. Angelika Mench-Hoinowski
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  4. Manfred Petersen
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Additional information

With support of Deutsche Forschungsgemeinschaft (SFB 33 and Schwerpunkt “Biochemische Humangenetik”)

With support of Deutsche Forschungsgemeinschaft (SFB 33)

Part of the results was obtained during M.D. thesis work of M.P.

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Langenbeck, U., Behbehani, A., Mench-Hoinowski, A. et al. Absence of a significant renal threshold for two aromatic acids in phenylketonuric children over two years of age. Eur J Pediatr 134, 115–118 (1980). https://doi.org/10.1007/BF01846027

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  • DOI: https://doi.org/10.1007/BF01846027

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