Efficacy and Safety of Odevixibat in Children With Biliary Atresia Who Have Undergone a Kasai HPE (BOLD)

Inclusion Criteria:

• A male or female patient with a clinical diagnosis of BA

• Age at Kasai HPE ≤90 days

• Eligible to start study treatment within 3 weeks post-Kasai HPE

Key Exclusion Criteria:

• Patients with intractable ascites

• Ileal resection surgery

• ALT ≥10× upper limit of normal (ULN) at screening

• Patients reliant only on total parenteral nutrition, or not able to take studymedication orally, at randomization

• Acute ascending cholangitis (patients may be randomized after resolution of acuteascending cholangitis)

• Choledochal cystic disease

• INR >1.6 (the patient may be treated with Vitamin K intravenously; sample may beredrawn and if INR is ≤1.6 at resampling the patient may be randomized)

• Any other conditions or abnormalities, including congenital abnormalities, majorcardiac surgery, hepatic, biliary, or GI disease which, in the opinion of theInvestigator or Medical Monitor, may compromise the safety of the patient, theintegrity of study results, or patient compliance with study requirements

• Weight <3.5kg at randomization