Indications: maintenance treatment of hyperammonemia in patients with urea cycle disorders involving deficiencies of the following enzymes; carbamoyl phosphate synthetase, ornitihine trascarbamylase, argininosuccinic acid synthetase, arginosuccinase, arginase, N-acetyglutamate synthetase.

Contra-indications: hypersensitivity, risk of kernicterus metabolic acidosis.

Side Effects: Nausea and vomiting overdose may include anorexia, irritability lethargy renal tubular dysfunction hypokalemia, hypocalcaemia, coma and metabolic acidosis.

Dose: Neonate – adult: 50mg-150mg /kg 3-4 times daily.

Maintenance,