nitisinone

Adult Dosing .

Dosage forms:  CAP: 2 mg, 5 mg, 10 mg, 20 mg

hereditary tyrosinemia, type 1

[1-2 mg/kg/dose PO qd]

Start: 0.5 mg/kg/dose PO bid, then incr. to 0.75 mg/kg/dose PO bid if detectable plasma and/or urine succinylacetone levels 4wk after tx start, then may give 1-2 mg/kg/dose PO qd if undetectable serum and urine succinylacetone levels after at least 4wk on current dose; Max: 2 mg/kg/day; Info: use in combo w/ dietary restriction of tyrosine, phenylalanine; give caps 1h before or 2h after meals; see pkg insert for dose adjustments

renal dosing

[not defined]

renal impairment: not defined

HD/PD: not defined

hepatic dosing

[not defined]

Peds Dosing .

Dosage forms:  CAP: 2 mg, 5 mg, 10 mg, 20 mg

hereditary tyrosinemia, type 1

[<5 yo]

Dose: 0.5-1 mg/kg/dose PO bid; Start: 0.5 mg/kg/dose PO bid, then incr. to 0.75 mg/kg/dose PO bid if detectable plasma and/or urine succinylacetone levels 4wk after tx start; Max: 2 mg/kg/day; Info: use in combo w/ dietary restriction of tyrosine, phenylalanine; give caps 1h before or 2h after meals; see pkg insert for dose adjustments

[5 yo and older]

Dose: 1-2 mg/kg/dose PO qd; Start: 0.5 mg/kg/dose PO bid, then incr. to 0.75 mg/kg/dose PO bid if detectable plasma and/or urine succinylacetone levels 4wk after tx start, then may give 1-2 mg/kg/dose PO qd if undetectable serum and urine succinylacetone levels after at least 4wk on current dose; Max: 2 mg/kg/day; Info: use in combo w/ dietary restriction of tyrosine, phenylalanine; give caps 1h before or 2h after meals; see pkg insert for dose adjustments

renal dosing

[not defined]

renal impairment: not defined

HD/PD: not defined

hepatic dosing

[not defined]