Abstract
An infant with propionic acidemia presented at one month of age with hyperammonemic coma. Treatment by two double-volume exchange transfusions did not have an appreciable effect, but hemodialysis led to a substantial reduction of the serum concentration of ammonia on two occasions. Nevertheless, continued therapy with sodium benzoate, arginine-HCl, carnitine and lactulose did not have any observable effect on the blood concentration of ammonia. Treatment with sodium phenylacetate was followed by a reduction in serum concentrations of ammonia to normal levels which were maintained. These observations demonstrate the dramatic reduction in serum concentrations of ammonia that may be obtained in patients with organic acidemia by hemodialysis. They suggest that pharmacologic acylation therapy with phenylacetate may be of lasting benefit in the management of this complication.
Original language | English (US) |
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Pages (from-to) | 87-96 |
Number of pages | 10 |
Journal | Journal of Neurogenetics |
Volume | 4 |
Issue number | 1 |
DOIs | |
State | Published - 1987 |
Externally published | Yes |
Keywords
- Hemodialysis
- Hyperammonemic coma
- Pharmacologic acylation
- Phenyl acetate
- Propionic acidemia
ASJC Scopus subject areas
- Genetics
- Cellular and Molecular Neuroscience