A Study to Evaluate the Safety and Tolerability of Maralixibat in Infant Participants With Cholestatic Liver Diseases Including Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille Syndrome (ALGS).

NCT ID: NCT04729751

Last Updated: 2025-02-06

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE2

Total Enrollment

27 participants

Study Classification

INTERVENTIONAL

Study Start Date

2021-09-09

Study Completion Date

2024-12-17

Brief Summary

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This study is designed to assess whether the investigational drug maralixibat, is safe and well tolerated in children \<12 months of age with Alagille Syndrome \[ALGS\] or Progressive Familial Intrahepatic Cholestasis \[PFIC\].

Detailed Description

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This is an open label study where all participants will receive maralixibat treatment.

Conditions

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Progressive Familial Intrahepatic Cholestasis Alagille Syndrome Cholestatic Liver Disease

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Single group with 2 cohorts - ≥ 6 participants each from ALGS and PFIC.
Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Maralixibat

Participants will receive up to 600 μg/kg twice daily (PFIC) or up to 400 μg/kg once daily (ALGS) over 13 weeks in the core study and for the duration of the Long Term Extension (LTE) where applicable.

Group Type EXPERIMENTAL

Maralixibat

Intervention Type DRUG

Maralixibat chloride provided in the form of an oral solution (i.e., 5, 10, 15, and 20 mg/mL)

* 400 μg/kg maralixibat chloride is equivalent to 380 µg/kg maralixibat free base
* 600 μg/kg maralixibat chloride is equivalent to 570 µg/kg maralixibat free base

Interventions

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Maralixibat

Maralixibat chloride provided in the form of an oral solution (i.e., 5, 10, 15, and 20 mg/mL)

* 400 μg/kg maralixibat chloride is equivalent to 380 µg/kg maralixibat free base
* 600 μg/kg maralixibat chloride is equivalent to 570 µg/kg maralixibat free base

Intervention Type DRUG

Other Intervention Names

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Formerly LUM001 and SHP625

Eligibility Criteria

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Inclusion Criteria

1. Body weight of ≥2.5 kg
2. \<12 months of age at the baseline visit (ROW). \>31 days and \<12 months of age at the baseline visit (US).
3. Gestational age ≥36 weeks at birth. For children born with gestational age between 32 and 36 weeks, a postmenstrual age of ≥36 weeks is required.
4. Diagnosis of PFIC or ALGS

Exclusion Criteria

1. Predicted complete absence of bile salt excretion pump (BSEP) function
2. History of surgical disruption of the enterohepatic circulation
3. History of liver transplant or imminent need for liver transplant
4. Decompensated cirrhosis
5. Presence of any other disease or condition known to interfere with the absorption, distribution, metabolism, or excretion of drugs, including bile salt metabolism in the intestine (e.g., inflammatory bowel disease), per investigator discretion
6. Presence of other significant liver disease or any other conditions or abnormalities which, in the opinion of the investigator or medical monitor, may compromise the safety of the participant or interfere with the participant's participation in or completion of the study
Minimum Eligible Age

0 Days

Maximum Eligible Age

364 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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Children Hospital LA

Los Angeles, California, United States

Site Status

University of California - San Francisco

San Francisco, California, United States

Site Status

Medstar Georgetown University Hospital

Washington D.C., District of Columbia, United States

Site Status

Ochsner Hospital for Children

New Orleans, Louisiana, United States

Site Status

Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

Cliniques Universitaires Saint-Luc

Brussels, , Belgium

Site Status

Sociedade Beneficente de Senhoras - Hospital Sírio-Libanês

São Paulo, , Brazil

Site Status

Hôpital Kremlin Bicêtre

Le Kremlin-Bicêtre, , France

Site Status

Hopital Necker

Paris, , France

Site Status

Consultorio de Joshue David Covarrubias Esquer

Zapopan, , Mexico

Site Status

Instytut Pomnik-Centrum Zdrowia Dziecka

Warsaw, , Poland

Site Status

King's College Hospital

London, , United Kingdom

Site Status

Countries

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United States Belgium Brazil France Mexico Poland United Kingdom

Related Links

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Other Identifiers

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2020-004628-40

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

MRX-801

Identifier Type: -

Identifier Source: org_study_id

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