Long-Term Safety Study of Maralixibat in Cholestatic Liver Disease

  • Research type

    Research Study

  • Full title

    MRX-800: A Long-Term Safety Study of Maralixibat, an Apical Sodium-Dependent Bile Acid Transporter Inhibitor (ASBTi), in the Treatment of Cholestatic Liver Disease in Subjects Who Previously Participated in a Maralixibat Study

  • IRAS ID

    274904

  • Contact name

    Alastair Baker

  • Contact email

    alastair.baker@nhs.net

  • Sponsor organisation

    Mirum Pharmaceutical Inc.

  • Eudract number

    2019-002755-42

  • ISRCTN Number

    ISRCTN00000000

  • Clinicaltrials.gov Identifier

    NCT00000000

  • Duration of Study in the UK

    4 years, 11 months, 23 days

  • Research summary

    Progressive familial intrahepatic cholestasis (PFIC) is a rare liver disease that primarily affects children. It is a disease where there is poor bile flow and substances are retained in the liver that would normally be excreted into bile. It tends to be passed down to a child from the parents, and gets worse over time. PFIC is associated with short life expectancy and devastating consequences on patients' quality of life.

    Alagille syndrome (ALGS) is an inherited cholestatic liver disease in children. Patients with ALGS have too much bile acid in their liver and body that can cause problems with the liver, and cause itching with or without jaundice.

    There is currently no approved treatment for PFIC or ALGS and available treatments have limited efficacy. Maralixibat is an investigational medicine (not yet approved by regulatory authorities) which will be given to participants twice a day as an oral solution. This study is a long-term follow up to previous maralixibat studies, designed to evaluate the long-term safety of maralixibat, and whether it will relieve or reduce symptoms and itching.

    Based on the high unmet medical need, the current absence of approved treatments and the complications with surgical treatment, the overall benign safety profile, and positive benefit-risk ratio of maralixibat show support for this long-term study. The study also aims to provide long-term access to maralixibat, particularly to those patients who benefited from the treatment in previous studies.

  • REC name

    London - Hampstead Research Ethics Committee

  • REC reference

    20/LO/0038

  • Date of REC Opinion

    19 Mar 2020

  • REC opinion

    Further Information Favourable Opinion