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Diagnostic value of orotic acid excretion in heritable disorders of the urea cycle and in hyperammonemia due to organic acidurias.

Abstract
Orotic acid excretion in urine in increased in ornithine transcarbamylase deficiency, citrullinemia and argininemia; it is barely increased in argininosuccinic aciduria and normal in carbamylphosphate synthetase deficiency and in hyperammonemia due to organic aciduria. The determination of orotic acid excretion is useful in differentiating the cases of hyperammonemia and reduces the need for enzymatic assays on tissue biopsies for decisions on therapy. The data indicate that orotic acid does not merely reflect ammonia concentration in plasma, but depends on carbamylphosphate concentration. Arginine could play a key role in the regulation of ammonia detoxication.
AuthorsC Bachmann, J P Colombo
JournalEuropean journal of pediatrics (Eur J Pediatr) Vol. 134 Issue 2 Pg. 109-13 (Aug 1980) ISSN: 0340-6199 [Print] Germany
PMID7439194 (Publication Type: Journal Article)
Chemical References
  • Citrulline
  • Orotic Acid
  • Ammonia
  • Arginine
Topics
  • Amino Acid Metabolism, Inborn Errors (urine)
  • Ammonia (blood)
  • Arginine (metabolism)
  • Child
  • Child, Preschool
  • Citrulline (metabolism)
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Metabolism, Inborn Errors (urine)
  • Middle Aged
  • Orotic Acid (urine)

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