Orotic Aciduria in Lysinuric Protein Intolerance: Dependence on the Urea Cycle Intermediates

@article{Rajantie1981OroticAI,
  title={Orotic Aciduria in Lysinuric Protein Intolerance: Dependence on the Urea Cycle Intermediates},
  author={Jukka Rajantie},
  journal={Pediatric Research},
  year={1981},
  volume={15},
  pages={115-119}
}
Summary: Urinary excretion of orotic acid was measured in controls and in subjects homozygous and heterozygous for lysinuric protein intolerance under various conditions of amino nitrogen intake. In all situations, the controls excreted less than 28 μg/kg/hr. Only one of the two heterozygotes studied differed from the controls. The orotic aciduria of homozygotes was normal in fasting but increased on a self-chosen low-protein diet (log mean, 80; range, 8 to 588 μg/kg/hr in 24-hr urine), after… CONTINUE READING

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