Niemann-Pick Type C Treatment With Adrabetadex for Symptoms of Brain and Nervous System

NCT ID: NCT03643562

Last Updated: 2024-01-17

Study Results

Results available

Outcome measurements, participant flow, baseline characteristics, and adverse events have been published for this study.

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Basic Information

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Recruitment Status

TERMINATED

Clinical Phase

PHASE3

Total Enrollment

27 participants

Study Classification

INTERVENTIONAL

Study Start Date

2018-06-18

Study Completion Date

2021-11-02

Brief Summary

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This study was amended from expanded access to a clinical trial. Information will be collected about long-term safety and effectiveness of adrabetadex shots in the spine every 2 weeks.

Participants who were already taking adrabetadex will receive their stable dose. Participants who have not ever taken it will start by receiving 400 mg.

Participants will receive treatment every 2 weeks until their doctor finds it does not help them anymore, they withdraw, or the study is stopped for any reason. Participants will not receive additional study treatment after their participation in this protocol.

Detailed Description

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Conditions

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Niemann-Pick Type C Disease

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Continued treatment for children at least 4 years of age
Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Adrabetadex

Participants will receive prescribed adrabetadex by intra-thecal (IT) injection every 2 weeks.

Group Type EXPERIMENTAL

Adrabetadex

Intervention Type DRUG

Administered via lumbar puncture (LP) and IT infusion

Interventions

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Adrabetadex

Administered via lumbar puncture (LP) and IT infusion

Intervention Type DRUG

Other Intervention Names

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VTS-270

Eligibility Criteria

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Inclusion Criteria

To be included in the study, a participant must meet the following criteria:

* Is male or female and at least 4 years of age at time of screening.
* Has a confirmed diagnosis of NPC and exhibits neurologic symptoms.
* Has written informed consent/assent to participate.
* Has the ability to undergo LP and IT drug administration.
* If taking miglustat (Zavesca®), must have been on a stable dose for the past 6 weeks and be willing to remain on a stable dose for the duration of participation in the study, or discontinue miglustat use at least 6 weeks before entry into the study (Day 1).
* If has a history of seizures, the condition is adequately controlled as per protocol requirements.
* Agrees to discontinue any investigational treatments (other than adrabetadex) for at least 1 month before first dose on Day 1.
* If engaging in heterosexual sex, agrees to use a protocol-defined method of contraception throughout the study, and until 30 days after completing the study.
* Has a responsible adult who the investigator determines is able and willing to comply with study requirements and a parent/guardian who will accompany the participant to study visits.

Exclusion Criteria

Participants will be excluded from the study if they meet any of the following criteria:

* Weighs less than 15 kg.
* Has a history of hypersensitivity reactions to any product containing 2-hydroxypropyl-β-cyclodextrin (HP-β-CD) or has a history of hypersensitivity reactions or allergy to anesthesia/sedation.
* Has received treatment with any investigational product (other than adrabetadex) within 1 month prior to Day 1 of treatment.
* Is pregnant or nursing.
* Has systemic infection or uncontrolled psychosis.
* Has known history of a bleeding disorder.
* Has used anticoagulants within 2 months of entry into the study.
* Per protocol, or in the opinion of the investigator:

1. has laboratory values that would preclude participation
2. has suspected infection of the central nervous system (CNS)
3. has a spinal deformity that could impact performance of repeated LPs
4. has a serious skin infection in the lumbar region or evidence of obstructive or normal pressure hydrocephalus
5. is unable to comply with the study requirements
6. has a medical condition that might increase the risk of participation
Minimum Eligible Age

4 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Mandos LLC

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Clinical Study Lead

Role: STUDY_DIRECTOR

Mandos LLC

Locations

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University of Arkansas System

Little Rock, Arkansas, United States

Site Status

Loma Linda University Health System

Loma Linda, California, United States

Site Status

Children's Hospital Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital of Orange County

Orange, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Children's National Hospital

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

Site Status

Rare Disease Research, LLC

Atlanta, Georgia, United States

Site Status

Rush University Medical Center

Chicago, Illinois, United States

Site Status

Gillette Children's Specialty Healthcare

Saint Paul, Minnesota, United States

Site Status

Children's Specialty Center of Nevada

Las Vegas, Nevada, United States

Site Status

NYU Langone Medical Center

New York, New York, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Oregon Health & Science University

Portland, Oregon, United States

Site Status

The Children's Hospital at TriStar Centennial

Nashville, Tennessee, United States

Site Status

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Site Status

Dell Children's Medical Center of Central Texas

Austin, Texas, United States

Site Status

Carilion Medical Center, Carilion Clinic

Roanoke, Virginia, United States

Site Status

Countries

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United States

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Other Identifiers

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VTS-270-001

Identifier Type: -

Identifier Source: org_study_id

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