Back to Search
Start Over
HYPERAMMONEMIA DUE TO A DEFECT IN HEPATIC ORNITHINE TRANSCARBAMYLASE.
- Source :
-
Pediatrics . Jul72, Vol. 50 Issue 1, p100. 12p. - Publication Year :
- 1972
-
Abstract
- A 9-year-old girl with vomiting, changes in behavior, coma, and evidence of hepatic dysfunction at 3 1/2 years of age was found to have hyperammonemia and decreased activity of liver ornithine transcarbamylase. When her dietary protein was reduced, she had improvement in her clinical condition and a return to normal of all hepatic function indices. Despite a defect in an enzyme of ornithine metabolism, she did not have hyperaminoacidemia, specifically hyperornithinemia, even when she had hyperammonemia. When she ingested a large amount of ornithine (300 mg/kg) she developed hyperornithinemia and hyperornithinuria. She also had orotic aciduria despite having normal activities of red cell orotidylic decarboxylase and pyrophosphorylase. Treatment with a low protein diet and citric acid supplements has been successful in preventing hyperammonemia and promoting growth and development. [ABSTRACT FROM AUTHOR]
- Subjects :
- *PHYSIOLOGICAL effects of ammonia
*ORNITHINE decarboxylase
*LIVER diseases
Subjects
Details
- Language :
- English
- ISSN :
- 00314005
- Volume :
- 50
- Issue :
- 1
- Database :
- Academic Search Index
- Journal :
- Pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 6723877
- Full Text :
- https://doi.org/10.1542/peds.50.1.100