From: Suggested guidelines for the diagnosis and management of urea cycle disorders
Disorder |
Sodium benzoate (to be given IV in 10% glucose) |
Sodium PBA/Sodium phenylacetate (to be given IV in 10% glucose) |
L-arginine hydrochloride^{a} (to be given IV in 10% glucose) |
N-carbamylglutamate (only oral/enteral drug) |
---|---|---|---|---|
Undiagnosed patient ^{b} | 250mg/kg as a bolus in 90–120 min, then: maintenance 250-500mg/kg/d ^{c} (if >20 kg body weight, 5.5 g/m2/d) | 250mg/kg as a bolus in 90–120 min, then maintenance: 250-500mg/kg/d ^{c} (1.2mmol/kg/d) | 250(−400) mg/kg (1-2mmol/kg) as a bolus in 90–120 min, then maintenance 250 mg/kg/d (1.2mmol/kg/d) | 100mg/kg bolus per NG tube then 25–62.5mg/kg every 6h |
NAGSD | same | – | 250 mg/kg (1.2mmol/kg) as a bolus in 90–120 min, then maintenance 250mg/kg/d (1.2mmol/kg/d) | same |
CPS1D & OTCD | same | 250mg/kg as bolus in 90–120 min, then maintenance: 250(−500) mg/kg/d ^{c} | same | – |
ASSD | same | same | same | – |
ASLD ^{d} | same | 250mg/kg as bolus in 90–120 min, then maintenance: 250mg/kg/d ^{c} | 200-400mg/kg (1-2mmol/kg) as bolus in 90–120 min, then maintenance 200-400mg/kg/d (1-2mmol/kg/d) | – |
ARG1D ^{e} | same | – | AVOID | – |
HHH syndrome | same | 250mg/kg as bolus in 90–120 min, then maintenance: 250mg/kg/d ^{c} | – | – |