Ravicti

Adult Dosing .

Dosage forms:  SOL: 1.1 g per mL

Restricted Distribution in US

[1-855-823-7878 or www.ravicti.com for more info]

chronic hyperammonemia, urea cycle disorder-assoc.

[initial tx]

Dose: 4.5-11.2 mL/m^2/day PO divided tid; Start: 0.6 mL per g dietary protein per day PO 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 w/ food; adjust dose based on pt clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate]

Dose: total daily PO dose (mL) = total daily sodium phenylbutyrate tab (g) x 0.86 = total daily sodium phenylbutyrate pwdr (g) x 0.81; Max: 17.5 mL/day; Info: divide doses tid and round each dose up to nearest 0.5 mL; give w/ food; adjust dose based on pt 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 divided tid

Peds Dosing .

Dosage forms:  SOL: 1.1 g per mL

Restricted Distribution in US

[1-855-823-7878 or www.ravicti.com for more info]

chronic hyperammonemia, urea cycle disorder-assoc.

[initial tx, <2 yo]

Dose: 4.5-11.2 mL/m^2/day PO divided tid; Start: 0.6 mL per g dietary protein per day PO 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 0.1 mL; give w/ food; adjust dose based on pt clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[initial tx, 2 yo and older]

Dose: 4.5-11.2 mL/m^2/day PO divided tid; Start: 0.6 mL per g dietary protein per day PO 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 w/ food; adjust dose based on pt clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate, <2 yo]

Dose: total daily PO dose (mL) = total daily sodium phenylbutyrate tab (g) x 0.86 = total daily sodium phenylbutyrate pwdr (g) x 0.81; Max: 17.5 mL/day; Info: divide doses tid and round each dose up to nearest 0.1 mL; give w/ food; adjust dose based on pt clinical response, plasma ammonia, and urinary phenylacetylglutamine levels

[conversion from sodium phenylbutyrate, 2 yo and older]

Dose: total daily PO dose (mL) = total daily sodium phenylbutyrate tab (g) x 0.86 = total daily sodium phenylbutyrate pwdr x 0.81; Max: 17.5 mL/day; Info: divide doses tid and round each dose up to nearest 0.5 mL; give w/ food; adjust dose based on pt 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 divided tid