Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.

@article{Enns2007SurvivalAT,
  title={Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.},
  author={Gregory M. Enns and Susan A Berry and Gerard Thomas Berry and William James Rhead and Saul W. Brusilow and Ada Hamosh},
  journal={The New England journal of medicine},
  year={2007},
  volume={356 22},
  pages={2282-92}
}
BACKGROUND The combination of intravenous sodium phenylacetate and sodium benzoate has been shown to lower plasma ammonium levels and improve survival in small cohorts of patients with historically lethal urea-cycle enzyme defects. METHODS We report the results of a 25-year, open-label, uncontrolled study of sodium phenylacetate and sodium benzoate therapy (Ammonul, Ucyclyd Pharma) in 299 patients with urea-cycle disorders in whom there were 1181 episodes of acute hyperammonemia. RESULTS… CONTINUE READING
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