Hyperammonemia in urea cycle disorders: role of the nephrologist.

  title={Hyperammonemia in urea cycle disorders: role of the nephrologist.},
  author={Robert S. Mathias and Dana R. Kostiner and Seymour Packman},
  journal={American journal of kidney diseases : the official journal of the National Kidney Foundation},
  volume={37 5},
Hyperammonemia associated with inherited disorders of amino acid and organic acid metabolism is usually manifested by irritability, somnolence, vomiting, seizures, and coma. Although the majority of these patients present in the newborn period, they may also present in childhood, adolescence, and adulthood with failure to thrive, persistent vomiting, developmental delay, or behavioral changes. Persistent hyperammonemia, if not treated rapidly, may cause irreversible neuronal damage. After the… CONTINUE READING