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Buphenyl
sodium phenylbutyrate
Adult Dosing .
Dosage forms: TAB: 500 mg; PWDR: 3 g per tsp, 8.6 g per Tbsp
chronic hyperammonemia, urea cycle disorder-assoc.
- [9.9-13 g/m^2/day PO divided 3-6x/day]
- Max: 20 g/day; Info: for pts w/ carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase deficiency; adjust dose based on pt clinical response and plasma ammonia levels; give w/ food; separate doses by at least 3h
renal dosing
- [see below]
- renal impairment: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [adjust dose amount]
- hepatic impairment: use lowest usual start dose
Peds Dosing .
- Dosage forms: TAB: 500 mg; PWDR: 3 g per tsp, 8.6 g per Tbsp
chronic hyperammonemia, urea cycle disorder-assoc.
- [<20 kg]
- Dose: 450-600 mg/kg/day PO divided 3-6x/day; Max: 20 g/day; Info: for pts w/ carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase deficiency; adjust dose based on pt clinical response and plasma ammonia levels; give w/ food; separate doses by at least 3h
- [>20 kg]
- Dose: 9.9-13 g/m^2/day PO divided 3-6x/day; Max: 20 g/day; Info: for pts w/ carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase deficiency; adjust dose based on pt clinical response and plasma ammonia levels; give w/ food; separate doses by at least 3h
renal dosing
- [see below]
- renal impairment: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [adjust dose amount]
- hepatic impairment: use lowest usual start dose