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An Open-label Extension Study to Evaluate Long-term Efficacy and Safety of Odevixibat in Children With Biliary Atresia

Phase 3
Conditions
Biliary Atresia
Interventions
Registration Number
NCT05426733
Lead Sponsor
Albireo, an Ipsen Company
Brief Summary

An Open-label Extension Study to Evaluate Long-term Efficacy and Safety of Odevixibat (A4250) in Children with Biliary Atresia

Detailed Description

This is a Phase 3, multi-center, open-label extension study to evaluate the long-term efficacy and safety of odevixibat in patients with Biliary Atresia (BA). Patients who completed treatment in the A4250-011 BOLD study and meet eligibility criteria for Study A4250-016 (BOLD-EXT) can participate. The duration of the treatment period is 104 weeks, followed by a 4-week Safety Follow-up Period. Patients who wish to continue receiving odevixibat after 104 weeks can remain on treatment in the optional extension period (OEP).

Up to 180 patients will be enrolled at approximately 70 sites in the North America, Europe, the Middle East and Asia-Pacific.

Recruitment & Eligibility

Status
ENROLLING_BY_INVITATION
Sex
All
Target Recruitment
180
Inclusion Criteria
  • Completion of the 104-week Treatment Period of Study A4250-011
  • Signed informed consent by caregiver
Exclusion Criteria
  • Patients who were not compliant with study drug treatment or procedures in Study A4250-011 as per the investigator's discretion
  • Any conditions or abnormalities which, in the opinion of the investigator, may compromise the safety of the patient, or interfere with the patient participating in or completing the study
  • Known hypersensitivity to any components of odevixibat
  • Patients who are scheduled for a liver transplant or are likely to require a liver transplant in the immediate future based on the investigator's judgment

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Odevixibat (A4250)OdevixibatCapsules for oral administration once daily for 104 weeks.
Primary Outcome Measures
NameTimeMethod
Proportion of patients who are alive and have not undergone liver transplantFrom baseline to Week 104
Secondary Outcome Measures
NameTimeMethod
Change in Fibrosis-4 (Fib-4) scoreFrom baseline to Week 104

The Fib-4 score provides an estimate of the amount of scarring (fibrosis) in the liver. A higher score indicates a greater amount of scarring in the liver.

* A score \< 1.45 indicates a low likelihood of fibrosis

* A score \> 3.25 indicates a higher probability of fibrosis

Change in heightFrom Baseline to Weeks 26, 52, 78 and 104
Change in weightFrom Baseline to Weeks 26, 52, 78 and 104
Change in serum bile acidsFrom Baseline to Weeks 26, 52, 78, and 104
Change in mid-arm circumferenceFrom Baseline to Weeks 26, 52, 78 and 104

Measure of growth

Change in pediatric end-stage liver disease (PELD) scoreFrom Baseline to Weeks 26, 52, 78 and 104

The PELD (pediatric end-stage liver disease) score is a measure of disease severity. PELD scores can range from a negative value (such as -10) to high numbers (such as 45). A higher score indicates more severe disease.

Change in aspartate aminotransferase (AST) to platelet ratio index (APRI) scoreFrom baseline to Week 104

The APRI (aspartate aminotransferase to platelet ratio index) score estimates the severity of liver fibrosis. A higher score indicates a greater amount of scarring (fibrosis) in the liver.

* A score ≤ 0.5 indicates a low likelihood of fibrosis

* A score ≥ 1.5 indicates a higher probability of fibrosis

Time to onset of any sentinel eventsFrom Baseline to Week 104

Trial Locations

Locations (30)

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Indiana University school of Medicine

🇺🇸

Indianapolis, Indiana, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

Guangzhou Women And Childrens Medical Center

🇨🇳

Guangdong, China

Children's Mercy Hospital and Clinics

🇺🇸

Kansas City, Missouri, United States

Royal Children's Hospital

🇦🇺

Parkville, Australia

Seattle Children's Hospital

🇺🇸

Seattle, Washington, United States

Children's Hospital of Fudan University

🇨🇳

Shanghai, China

Hospital Universitari Vall d'Hebron

🇪🇸

Barcelona, Spain

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

The Childrens Hospital at Montefiore Albert Einstein School of Medicine

🇺🇸

Bronx, New York, United States

Children's Healhcare of Atlanta- Emory University school of medicine

🇺🇸

Atlanta, Georgia, United States

Hassenfeld Children's Hospital at NYU Langone

🇺🇸

New York, New York, United States

Columbia University Medical Center New York Presbyterian Morgan Stanley-Komansky Childrens Hospital

🇺🇸

New York, New York, United States

Instytut Pomnik-Centrum Zdrowia Dziecka

🇵🇱

Warsaw, Poland

University Malaya Medical Centre

🇲🇾

Kuala Lumpur, Malaysia

Starship Child Health, Te Whatu Ora - Health New Zealand

🇳🇿

Auckland, New Zealand

University Children´s Hospital Tuebingen

🇩🇪

Tuebingen, Germany

Hospital Raja Perempuan Zainab II

🇲🇾

Kota Bharu, Malaysia

University Medical Center Groningen

🇳🇱

Groningen, Netherlands

Akdeniz University Medical Faculty

🇹🇷

Antalya, Turkey

Seoul National University Childrens Hospital

🇰🇷

Seoul, Korea, Republic of

The Hospital for Sick Children

🇨🇦

Toronto, Canada

Bicêtre Hospital

🇫🇷

Le Kremlin-Bicêtre, France

Hacettepe İhsan Doğramacı Children's Hospital

🇹🇷

Ankara, Turkey

Istanbul University Istanbul Medical Faculty Hospital

🇹🇷

Istanbul, Turkey

CHU Sainte-Justine

🇨🇦

Montréal, Canada

ASST Papa Giovanni XXIII

🇮🇹

Bergamo, Italy

Azienda Ospedaliera di Padova

🇮🇹

Padova, Italy

Charité - Universitätsmedizin Berlin

🇩🇪

Berlin, Germany

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