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Diagnostic value of orotic acid excretion in heritable disorders of the urea cycle and in hyperammonemia due to organic acidurias
- Source :
- European Journal of Pediatrics; August 1980, Vol. 134 Issue: 2 p109-113, 5p
- Publication Year :
- 1980
-
Abstract
- Orotic acid excretion in urine is 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 causes 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.
Details
- Language :
- English
- ISSN :
- 03406199 and 14321076
- Volume :
- 134
- Issue :
- 2
- Database :
- Supplemental Index
- Journal :
- European Journal of Pediatrics
- Publication Type :
- Periodical
- Accession number :
- ejs17007142
- Full Text :
- https://doi.org/10.1007/BF01846026