From: Suggested guidelines for the diagnosis and management of urea cycle disorders
Disorder | Sodium benzoatea | Sodium PBAa,b | L-Argininea (hydrochloride or free base) | L-Citrullinea | Carbamyl- glutamatea |
---|---|---|---|---|---|
NAGSD | – | – | – | – | 10–100 mg/kg/d |
CPS1D | ≤ 250mg/kg/dc,d maximum: 12g/d | <20 kg: ≤250mg/kg/dc,d >20 kg: 5g/m2/d maximum: 12g/day | <20 kg: 100-200mg/kg/dc(0.5-1mmol/kg/d) >20 kg: 2.5-6g/m2/d maximum: 6g/d | 100-200mg/kg/de maximum: 6g/d | – |
OTCD | same | same | same | same | – |
ASSD | same | same | <20 kg: 100-300mg/kg/dc,d>20 kg: 2.5-6g/m2/d maximum: 6g/d | – | – |
ASLD | same | – | same | – | – |
ARG1D | same | same | – | – | – |
HHH syndrome | same | same | <20 kg: 100-200mg/kg/dc >20 kg: 2.5-6g/m2/d maximum: 6g/d | same | – |