Odevixibat

UNITS ARE MICROGRAMS PER KG (mcg/kg), NOT mg/kg. PFIC: if there is no improvement in pruritus after 3 months, increase in 40 micrograms/kg increments up to 120 micrograms/kg once daily (max 6 mg/day). ALGS tolerability: dose reduction to 40 micrograms/kg/day may be considered if tolerability issues occur in the absence of other causes; once stabilised, increase back to 120 micrograms/kg/day. Label weight-band tables — PFIC (40 mcg/kg/day): 7.4 kg and below 200 mcg; 7.5-12.4 kg 400 mcg; 12.5-17.4 kg 600 mcg; 17.5-25.4 kg 800 mcg; 25.5-35.4 kg 1,200 mcg; 35.5-45.4 kg 1,600 mcg; 45.5-55.4 kg 2,000 mcg; 55.5 kg and above 2,400 mcg. ALGS (120 mcg/kg/day), same weight bands: 600, 1,200, 1,800, 2,400, 3,600, 4,800, 6,000 and 7,200 mcg respectively. Take at least 4 hours before or 4 hours after a bile acid binding resin. Establish the baseline pattern of liver test variability before starting and monitor ALT, AST, total and direct bilirubin and INR during treatment; interrupt if new-onset liver test abnormalities or symptoms of clinical hepatitis occur, and discontinue permanently if a hepatic decompensation event occurs. Geriatric: PFIC and ALGS are largely diseases of paediatric and young adult patients and clinical studies did not include patients 65 years and older. SOURCE CAVEAT: no UK SPC section was captured in the bundle; the regimen above is from the US prescribing information for Bylvay and must be verified against the UK SPC before publication.

UNIT IS MICROGRAMS/kg, NOT mg/kg. The 40 micrograms/kg once-daily figure is the PFIC dose for patients aged 3 months and older. For Alagille syndrome in patients aged 12 months and older the recommended dosage is 120 micrograms/kg once daily. Safety and effectiveness are not established below 3 months of age (PFIC) or below 12 months of age (ALGS). US labelling — verify against the UK SPC and a children's formulary before use.