Table 6

Dosages of commonly used drugs in acute hyperammonaemic decompensations in undiagnosed adult patients

DrugInitial dose (bolus)Maintenance dose
Sodium benzoate*250 mg/kg as bolus in 90–120 min250–500 mg/kg/day >20 kg body weight: 5.5 g/m2/day
Sodium PBA/sodium
phenylacetate*		250 mg/kg as bolus in 90–120 min250–500 mg/kg/day
L-arginine
Hydrochloride*		250–400 mg/kg (1–2 mmol/kg) bolus in
90–120 min		250 mg/kg/day (1.2 mmol/kg/day)
N-carbamylglutamate†100 mg/kg bolus per NG tube25–62.5 mg/kg every 6 hours

  • Table is adapted from Häberle et al.66

  • *Sodium benzoate, sodium phenylbutyrate (sodium PBA)/sodium phenylacetate, L-arginine hydrochloride to be given intravenously in glucose 10%. Nasogastric tube (NG).

  • †Only available as oral/enteral drug