A Safety Study of NNZ-2566 in Pediatric Rett Syndrome

NCT ID: NCT02715115

Last Updated: 2020-08-14

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

82 participants

Study Classification

INTERVENTIONAL

Study Start Date

2016-03-31

Study Completion Date

2017-01-05

Brief Summary

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The purpose of this study is to determine whether NNZ-2566 is safe and well tolerated in the treatment of Rett syndrome in children and adolescents.

Detailed Description

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Rett syndrome is a neurodevelopmental disorder primarily affecting females. The disorder is characterized by apparent normal development in early infancy (6-18 months), followed by a period of regression with onset of systemic and neurological signs. The CNS symptoms of Rett syndrome include learning disability, autism symptomatology and epilepsy and these can be severe and highly debilitating. Affected individuals also show signs of autonomic dysfunction, reflected in cardiovascular and respiratory abnormalities. There is no currently effective treatment for Rett syndrome.

This study will investigate the safety, tolerability and blood pharmacokinetics of treatment with oral administration of NNZ-2566 at 50 mg/kg, 100 mg/kg, 200 mg/kg BID, or placebo BID, in children and adolescent females with Rett syndrome. The study also will also investigate measures of efficacy and biomarkers during treatment.

Conditions

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Rett Syndrome

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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NNZ-2566

Glycyl-L-2-Methylpropyl-L-Glutamic Acid

Group Type EXPERIMENTAL

NNZ-2566

Intervention Type DRUG

Glycyl-L-2-Methylpropyl-L-Glutamic Acid (NNZ-2566) supplied as a lyophilized powder for reconstitution with strawberry flavored solution 0.5% v/v in Water for Injection.

Placebo (strawberry flavored solution)

Strawberry flavored solution and Water for Injection

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Strawberry flavored solution and Water for Injection

Interventions

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NNZ-2566

Glycyl-L-2-Methylpropyl-L-Glutamic Acid (NNZ-2566) supplied as a lyophilized powder for reconstitution with strawberry flavored solution 0.5% v/v in Water for Injection.

Intervention Type DRUG

Placebo

Strawberry flavored solution and Water for Injection

Intervention Type DRUG

Other Intervention Names

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trofinetide Strawberry flavoring

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of classic/typical Rett syndrome with a documented mutation of the MeCP2 gene.
* Age 5 - 15 years.
* Weight at Screening and Baseline between 15.0 kg-100.0 kg (at least 15.0 kg and no greater than 100.0 kg).
* Each subject must be able to swallow the study medication provided as a liquid solution, or via gastrostomy tube.

Exclusion Criteria

* Actively undergoing neurological regression
* Abnormal QT interval, prolongation or significant cardiovascular history.
* Current treatment with insulin.
* Anti-convulsants with liver enzyme inducing effects.
* Unstable seizure profile.
* Excluded concomitant medications.
* Current clinically significant (as determined by the investigator). cardiovascular, renal, hepatic, or respiratory disease.
* Gastrointestinal disease which may interfere with the absorption, distribution, metabolism or excretion of the study medication.
* History of, or current cerebrovascular disease or brain trauma.
* History of, or current clinically significant endocrine disorder, e.g. hypo- or hyperthyroidism, or diabetes mellitus.
* History of, or current, malignancy.
* Significant hearing and/or visual impairments that may affect ability to complete the test procedures.
* Allergy to strawberry.
Minimum Eligible Age

5 Years

Maximum Eligible Age

15 Years

Eligible Sex

FEMALE

Accepts Healthy Volunteers

No

Sponsors

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rettsyndrome.org

UNKNOWN

Sponsor Role collaborator

Neuren Pharmaceuticals Limited

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Daniel Glaze, MD

Role: PRINCIPAL_INVESTIGATOR

Baylor College of Medicine

Alan Percy, MD

Role: PRINCIPAL_INVESTIGATOR

University of Alabama at Birmingham

Timothy Feyma, MD

Role: PRINCIPAL_INVESTIGATOR

Gillette Children's Specialty Healthcare

Peter Heydemann, MD

Role: PRINCIPAL_INVESTIGATOR

Rush University Medical Center

Jeff Neul, MD

Role: PRINCIPAL_INVESTIGATOR

University of California, San Diego

Tim Benke, MD

Role: PRINCIPAL_INVESTIGATOR

Children's Hospital Colorado

Mary Jones, MD

Role: PRINCIPAL_INVESTIGATOR

UCSF Benioff Children's Hospital Oakland

Steve Skinner, MD

Role: PRINCIPAL_INVESTIGATOR

Greenwood Genetic Center

Mustafa Sahin, MD

Role: PRINCIPAL_INVESTIGATOR

Boston Children's Hospital

Sarika Peters, PhD

Role: PRINCIPAL_INVESTIGATOR

Vanderbilt University

Shannon Standridge

Role: PRINCIPAL_INVESTIGATOR

Children's Hospital Medical Center, Cincinnati

Eric Marsh, MD

Role: PRINCIPAL_INVESTIGATOR

Children's Hospital of Philadelphia

Locations

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University of Alabama

Birmingham, Alabama, United States

Site Status

UCSF Benioff Children's Hospital Oakland

Oakland, California, United States

Site Status

University of California, San Diego

San Diego, California, United States

Site Status

Children's Hosptial Colorado

Aurora, Colorado, United States

Site Status

Rush University Medical Center

Chicago, Illinois, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Gillette Children's Specialty Healthcare

Saint Paul, Minnesota, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Greenwood Genetic Center

Greenwood, South Carolina, United States

Site Status

Vanderbilt University

Nashville, Tennessee, United States

Site Status

Baylor College of Medicine

Houston, Texas, United States

Site Status

Countries

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United States

References

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Darwish M, Passarell J, Youakim JM, Bradley H, Bishop KM. Exposure-Response Efficacy Modeling to Support Trofinetide Dosing in Individuals with Rett Syndrome. Adv Ther. 2024 Apr;41(4):1462-1480. doi: 10.1007/s12325-024-02796-y. Epub 2024 Feb 16.

Reference Type DERIVED
PMID: 38363467 (View on PubMed)

Parent H, Ferranti A, Niswender C. Trofinetide: a pioneering treatment for Rett syndrome. Trends Pharmacol Sci. 2023 Oct;44(10):740-741. doi: 10.1016/j.tips.2023.06.008. Epub 2023 Jul 16.

Reference Type DERIVED
PMID: 37460385 (View on PubMed)

Other Identifiers

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Neu-2566-RETT-002

Identifier Type: -

Identifier Source: org_study_id

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