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Study to Assess Efficacy, Safety, Tolerability, Pharmacokinetics (PK), and Pharmacodynamics (PD) of Obeticholic Acid (OCA) Compared to Placebo in Pediatric Participants With Biliary Atresia, Post-hepatoportoenterostomy

Phase 2
Recruiting
Conditions
Biliary Atresia
Interventions
Drug: Matching Placebo
Registration Number
NCT06121375
Lead Sponsor
Intercept Pharmaceuticals
Brief Summary

This study will evaluate the efficacy, safety and tolerability, as well as PK/PD of OCA in eligible pediatric participants with biliary atresia with successful hepatoportoenterostomy (HPE, also known as a Kasai portoenterostomy). The double-blind period comprises of 2 phases: dose titration phase and age expansion treatment phase.

Detailed Description

Not available

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
144
Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Participants receiving Matching placeboMatching PlaceboParticipants will be randomized to receive matching placebo orally, with water, once daily. Dose will be titrated every 2 weeks in a stepwise manner for the first 6 weeks, starting at 1.5 mg AED and titrating through 3 mg AED to a maximum of 5 mg AED, as tolerated; a discussion with the Medical Monitor is encouraged when determining uptitration if considerable signs or symptoms have arisen. Following the 6-week dose titration phase, participants will continue at the tolerated dose for approximately 24 months in Age Expansion Treatment Phase.
Participants receiving OCAOCAParticipants will be randomized to receive OCA (starting at 1.5 milligrams \[mg\] adult equivalent dose \[AED\]) orally, with water, once daily. Dose will be titrated every 2 weeks in a stepwise manner for the first 6 weeks, starting at 1.5 mg AED and titrating through 3 mg AED to a maximum of 5 mg AED, as tolerated; a discussion with the Medical Monitor is encouraged when determining uptitration if considerable signs or symptoms have arisen. Following the 6-week dose titration phase, participants will continue at the tolerated dose for approximately 24 months in Age Expansion Treatment Phase.
Primary Outcome Measures
NameTimeMethod
Time to the First Occurrence of Composite EndpointUp to Week 64

To evaluate the effect of OCA compared to placebo in conjunction with established local standard of care on clinical outcomes in participants with biliary atresia who have had a successful Kasai procedure as measured by time to first occurrence of any of the following adjudicated events, derived as composite event endpoint of all-cause death, liver transplant, Pediatric end-stage liver disease (PELD) score ≥17/model of end-stage liver disease (MELD)≥15, Hospitalization (as defined by a stay of 24 hours or greater) for new onset or recurrence of Variceal bleed, hepatic encephalopathy (as defined by a West Haven score of ≥2), Spontaneous bacterial peritonitis (confirmed by diagnostic paracentesis) and Clinically evident ascites related to poral hypertension (diuretic-resistant ascites requiring therapeutic paracentesis at a frequency of at least twice in a month)

Secondary Outcome Measures
NameTimeMethod
Plasma concentration of unconjugated OCA (parent), glyco-OCA, tauro-OCA, and total OCA (molar sum of OCA and its active conjugates)Up to Week 64

Plasma concentrations of OCA and its conjugates (glyco-OCA and tauro-OCA) will be determined using validated liquid-chromatography mass spectrometry/mass spectrometry methods

Change from Baseline in Gamma Glutamyl Transferase (GGT)Baseline and up to Week 64

Blood samples will be calculated to assess GGT levels.

Change from Baseline in total and direct (conjugated) bilirubinBaseline and up to Week 64

Blood samples will be calculated to assess total and direct (conjugated) bilirubin levels.

Change from Baseline in Fibroblast Growth Factor-19 (FGF-19)Baseline and up to Week 64

Blood samples will be calculated to assess FGF-19

Change from Baseline in liver stiffness as assessed by transient elastographyBaseline and up to Week 64
Safety and tolerability as assessed by the incidence of treatment-emergent adverse events (TEAEs) including serious adverse events (SAEs)Up to Week 64
Change from Baseline in 7-hydroxyl-4-cholesten-3-one (C4)Baseline and up to Week 64

Blood samples will be calculated to assess C4

Change from Baseline in endogenous bile acidsBaseline and up to Week 64

Blood samples will be calculated to assess endogenous bile acids

Change from Baseline in plasma levels of fat-soluble vitamins (D and K)Baseline and up to Week 64

Blood samples will be calculated to assess plasma levels of fat-soluble vitamins (D and K)

Trial Locations

Locations (9)

Queensland Childrens Hospital

🇦🇺

South Brisbane, Queensland, Australia

Women's and Children's Hospital

🇦🇺

North Adelaide, South Australia, Australia

Stollery Children's Hospital

🇨🇦

Edmonton, Alberta, Canada

Queen Mary Hospital

🇭🇰

Hong Kong, Hong Kong

KK Women's and Children's Hospital

🇸🇬

Singapore, Singapore

Royal Childrens Hospital

🇦🇺

Parkville, Victoria, Australia

Hospital Raja Perempuan Azinab II

🇲🇾

Kota Bharu, Kelantan, Malaysia

University Malaya Medical Center

🇲🇾

Kuala Lumpur, Malaysia

Starship Child Health

🇳🇿

Auckland, New Zealand

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