Evaluation of Maralixibat in Biliary Atresia Response Post-Kasai

NCT ID: NCT04524390

Last Updated: 2025-03-19

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE2

Total Enrollment

75 participants

Study Classification

INTERVENTIONAL

Study Start Date

2021-07-08

Study Completion Date

2024-02-07

Brief Summary

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A study to evaluate the efficacy and safety of maralixibat in infants with Biliary Atresia (BA) after Hepatoportoenterostomy (HPE, also known as the Kasai procedure).

Detailed Description

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This is a double-blind randomized, placebo-controlled study in subjects with Biliary Atresia with a primary endpoint at Week 26 followed by long-term open-label period during which all subjects will receive maralixibat to Week 104.

Conditions

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Biliary Atresia

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

TRIPLE

Participants Caregivers Investigators

Study Groups

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Double Blind - Maralixibat

The double-blind period comprised of 4-8 weeks of dose escalation followed by 18 - 22 weeks of stable dosing treatment, after which participants were transferred to the open-label arm.

Group Type EXPERIMENTAL

Maralixibat

Intervention Type DRUG

A small molecule inhibitor of the ileal bile acid transporter (IBAT)

Double Blind - Placebo

The double-blind period comprised of 4-8 weeks of dose escalation followed by 18 - 22 weeks of stable dosing treatment, after which participants were transferred to the open-label arm.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type OTHER

Identical to maralixibat except for the active drug substance

Open Label - Maralixibat

The Open-Label period comprised of 4-8 weeks of dose escalation followed by 70 - 74 weeks of stable dosing treatment. During the OLE, all participants, regardless of treatment assignment in the double-blind period, received maralixibat.

Group Type EXPERIMENTAL

Maralixibat

Intervention Type DRUG

A small molecule inhibitor of the ileal bile acid transporter (IBAT)

Interventions

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Maralixibat

A small molecule inhibitor of the ileal bile acid transporter (IBAT)

Intervention Type DRUG

Placebo

Identical to maralixibat except for the active drug substance

Intervention Type OTHER

Other Intervention Names

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Formerly LUM001 and SHP625

Eligibility Criteria

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Inclusion Criteria

1. Male or female subjects with body weight ≥2500 g, who are ≥21 days old and \<90 days old at the time of HPE (Kasai)
2. HPE or Kasai Procedure within 3 weeks prior to randomization
3. Clinical diagnosis of biliary atresia

Exclusion Criteria

1. Subjects with intractable chronic diarrhea at randomization
2. Subjects not tolerating enteral feeds at randomization
3. History of ileal resection
4. Diagnosis of biliary atresia splenic malformation syndrome or cystic biliary atresia
5. Evidence of another non-biliary atresia pathology involving the intrahepatic bile duct (e.g., paucity, sclerosing cholangitis)
6. Evidence of liver failure (e.g. significant ascites)
Minimum Eligible Age

21 Days

Maximum Eligible Age

111 Days

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Mirum Pharmaceuticals, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Phoenix Children's Division of Gastroenterology & Hepatology

Phoenix, Arizona, United States

Site Status

Children's Healthcare of Atlanta - Emory University School of Medicine

Atlanta, Georgia, United States

Site Status

NYU Grossman School of Medicine

New York, New York, United States

Site Status

New York-Presbyterian - Columbia University Medical Center

New York, New York, United States

Site Status

Montefiore Medical Center

The Bronx, New York, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

Beijing Pediatric Research Institute

Beijing, Beijing Municipality, China

Site Status

Guangzhou Women and Children's Medical Center

Guangzhou, Guangdong, China

Site Status

The Children's Hospital, Zhejiang University School of Medicine

Hanzhou, Zhejiang, China

Site Status

Children's hospital of Shanghai

Shanghai, , China

Site Status

Children's Hospital of Fudan University

Shanghai, , China

Site Status

Hannover Medical School

Hanover, , Germany

Site Status

Instytut Pomnik-Centrum Zdrowia Dziecka

Warsaw, , Poland

Site Status

KK women's and Children's hospital

Bukit Timah, , Singapore

Site Status

Taichung Veterans General Hospital

Taichung, , Taiwan

Site Status

Linkou Chang Gung Memorial Hospital

Taoyuan District, , Taiwan

Site Status

Birmingham Children's Hospital

Birmingham, , United Kingdom

Site Status

King's College Hospital NHS

London, , United Kingdom

Site Status

Hue Central Hospital

Huế, Thừa Thiên Huế Province, Vietnam

Site Status

Vietnam National Children's Hospital

Hanoi, , Vietnam

Site Status

Children's Hospital No. 1

Ho Chi Minh City, , Vietnam

Site Status

Countries

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United States China Germany Poland Singapore Taiwan United Kingdom Vietnam

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

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MRX-701

Identifier Type: -

Identifier Source: org_study_id

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