Ravicti

Adult Dosing .

Dosage forms:  SOLUTION: 1.1 g per mL

chronic hyperammonemia, urea cycle disorder-associated

[phenylbutyrate-naive patients]

Dose: 4.5-11.2 mL/m^2/day PO/NG/GT divided tid; Start: 0.6 mL per g dietary protein per day PO/NG/GT divided tid; Max: 17.5 mL/day; Info: start 4.5 mL/m^2/day if residual enzyme activity; round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate tab]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.86] PO/NG/GT divided tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate powder]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.81] PO/NG/GT divided tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

renal dosing

[see below]

renal impairment: not defined, caution advised

HD/PD: not defined

hepatic dosing

[adjust dose amount]

Child-Pugh Class B or C: start 4.5 mL/m^2/day

Peds Dosing .

Dosage forms:  SOLUTION: 1.1 g per mL

chronic hyperammonemia, urea cycle disorder-associated

[phenylbutyrate-naive patients, <2 yo]

Dose: 4.5-11.2 mL/m^2/day PO/NG/GT divided at least tid; Start: 0.6 mL per g dietary protein per day PO/NG/GT divided at least tid; Max: 17.5 mL/day; Info: start 4.5 mL/m^2/day if residual enzyme activity; round each dose up to nearest 0.1 mL; give before breastfeeding or with food/formula; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[phenylbutyrate-naive patients, 2 yo and older]

Dose: 4.5-11.2 mL/m^2/day PO/NG/GT divided tid; Start: 0.6 mL per g dietary protein per day PO/NG/GT divided tid; Max: 17.5 mL/day; Info: start 4.5 mL/m^2/day if residual enzyme activity; round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate tab, <2 yo]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.86] PO/NG/GT divided at least tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.1 mL; give before breastfeeding or with food/formula; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate powder, <2 yo]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.81] PO/NG/GT divided at least tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.1 mL; give before breastfeeding or with food/formula; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate tab, 2 yo and older]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.86] PO/NG/GT divided tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate powder, 2 yo and older]

Dose: [total daily glycerol phenylbutyrate dose (mL) = total daily sodium phenylbutyrate dose (g) x 0.81] PO/NG/GT divided tid; Max: 17.5 mL/day; Info: round each dose up to nearest 0.5 mL; give with food; adjust dose based on clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

renal dosing

[see below]

renal impairment: not defined, caution advised

HD/PD: not defined

hepatic dosing

[adjust dose amount]

Child-Pugh Class B or C: start 4.5 mL/m^2/day