NCT04422431

Brief Summary

The main objective of the study is to evaluate the change in liver copper (Cu) concentration following 48 weeks of treatment with ALXN1840 in adult participants with Wilson Disease (WD) who have been previously treated for at least 1 year with standard of care (that is, trientine, penicillamine, or zinc). In the Treatment Period, efficacy and safety of ALXN1840 will be assessed at Week 48.

Trial Health

93
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
31

participants targeted

Target at P25-P50 for phase_2

Timeline
Completed

Started Dec 2020

Geographic Reach
8 countries

12 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

May 22, 2020

Completed
18 days until next milestone

First Posted

Study publicly available on registry

June 9, 2020

Completed
6 months until next milestone

Study Start

First participant enrolled

December 2, 2020

Completed
1.5 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 21, 2022

Completed
12 months until next milestone

Study Completion

Last participant's last visit for all outcomes

May 17, 2023

Completed
2 months until next milestone

Results Posted

Study results publicly available

July 3, 2023

Completed
Last Updated

October 18, 2024

Status Verified

October 1, 2024

Enrollment Period

1.5 years

First QC Date

May 22, 2020

Results QC Date

May 18, 2023

Last Update Submit

October 16, 2024

Conditions

Keywords

CopperALXN1840HistologyLiver Biopsy

Outcome Measures

Primary Outcomes (1)

  • Change From Baseline in Liver Cu Concentration at Week 48 (Treatment Period)

    Liver biopsy samples were taken for the assessment of liver Cu concentration. Multiple imputation was used to impute missing data at Week 48 due to any reason based on Baseline values.

    Baseline, Week 48 (Treatment Period)

Secondary Outcomes (15)

  • Number of Participants With Change From Baseline in Nonalcoholic Steatohepatitis Clinical Research Network (NASH CRN) Fibrosis Stage at Week 48 (Treatment Period)

    Baseline, Week 48 (Treatment Period)

  • Number of Participants With Change From Baseline in Metavir Fibrosis Score at Week 48 (Treatment Period)

    Baseline, Week 48 (Treatment Period)

  • Number of Participants With Change From Baseline in Ishak Fibrosis Score at Week 48 (Treatment Period)

    Baseline, Week 48 (Treatment Period)

  • Change From Baseline in Hepatic Collagen Content at Week 48 (Treatment Period)

    Baseline, Week 48 (Treatment Period)

  • Change From Baseline in a-SMA Content at Week 48 (Treatment Period)

    Baseline, Week 48 (Treatment Period)

  • +10 more secondary outcomes

Study Arms (1)

ALXN1840

EXPERIMENTAL

Participants will receive ALXN1840.

Drug: Bis-Choline Tetrathiomolybdate

Interventions

Participants will be initiated at 15 milligrams once daily, then the dose will be increased to 30 milligrams once daily at Week 6.

Also known as: ALXN1840
ALXN1840

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Diagnosis of WD by Leipzig Criteria ≥ 4 or by historical test results.
  • Continuous treatment for WD with penicillamine, trientine or zinc for at least 1 year prior to screening.
  • Body mass index \< 30 kilograms/meter squared.
  • Able to cooperate with a percutaneous liver biopsy.
  • Willing and able to follow protocol-specified contraception requirements.
  • Capable of giving signed informed consent.

You may not qualify if:

  • Decompensated cirrhosis or Model for End Stage Liver Disease score \> 13.
  • Modified Nazer score \> 7.
  • Clinically significant gastrointestinal bleed within past 3 months.
  • Alanine aminotransferase \> 2 × upper limit of normal.
  • History of bleeding abnormality or known coagulopathy, including platelet count \< 100,000, and international normalized ratio for prothrombin time ≥ 1.5.
  • Participant unwilling to accept blood products, if required.
  • Marked neurological disease requiring either nasogastric feeding tube or intensive inpatient medical care.
  • Hemoglobin less than lower limit of the reference range for age and sex.
  • Participants in renal failure, defined as in end-stage renal disease on dialysis (chronic kidney disease 5) or creatinine clearance \< 30 milliliters/minute.
  • Lymphoma, leukemia, or any malignancy within the past 5 years.
  • Current or chronic history of liver disease not associated with WD.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (12)

Research Site

Sacramento, California, 95817, United States

Location

Research Site

Ann Arbor, Michigan, 48109, United States

Location

Research Site

Dallas, Texas, 75235, United States

Location

Research Site

Toronto, Ontario, M5G 2C4, Canada

Location

Research Site

Århus N, 8200, Denmark

Location

Research Site

Grafton, 1010, New Zealand

Location

Research Site

Moscow, 119021, Russia

Location

Research Site

Saint Petersburg, 194017, Russia

Location

Research Site

Singapore, 169608, Singapore

Location

Research Site

Barcelona, 08036, Spain

Location

Research Site

Valencia, 46026, Spain

Location

Research Site

London, SE5 9RS, United Kingdom

Location

Related Links

MeSH Terms

Conditions

Hepatolenticular Degeneration

Interventions

tetrathiomolybdate

Condition Hierarchy (Ancestors)

Liver DiseasesDigestive System DiseasesBasal Ganglia DiseasesBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesBrain Diseases, Metabolic, InbornBrain Diseases, MetabolicMovement DisordersHeredodegenerative Disorders, Nervous SystemNeurodegenerative DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolism, Inborn ErrorsMetal Metabolism, Inborn ErrorsMetabolic DiseasesNutritional and Metabolic Diseases

Results Point of Contact

Title
Alexion Pharmaceuticals, Inc.
Organization
Alexion Pharmaceuticals, Inc.

Study Officials

  • Eugene S. Swenson, MD, PhD

    Alexion Pharmaceuticals, Inc.

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NON RANDOMIZED
Masking
NONE
Masking Details
This study is only pathologist-blinded for the assessments of liver histology samples.
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

May 22, 2020

First Posted

June 9, 2020

Study Start

December 2, 2020

Primary Completion

May 21, 2022

Study Completion

May 17, 2023

Last Updated

October 18, 2024

Results First Posted

July 3, 2023

Record last verified: 2024-10

Data Sharing

IPD Sharing
Will not share

Locations