NCT03636438

Brief Summary

Determine the long-term safety of DTX301 following a single intravenous (IV) dose in adults with late-onset ornithine transcarbamylase (OTC) deficiency.

Trial Health

82
On Track

Trial Health Score

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

Enrollment
11

participants targeted

Target at below P25 for all trials

Timeline
43mo left

Started Aug 2018

Longer than P75 for all trials

Geographic Reach
5 countries

9 active sites

Status
active not recruiting

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

Study Progress68%
Aug 2018Dec 2029

First Submitted

Initial submission to the registry

August 15, 2018

Completed
2 days until next milestone

First Posted

Study publicly available on registry

August 17, 2018

Completed
13 days until next milestone

Study Start

First participant enrolled

August 30, 2018

Completed
11.3 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2029

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

December 1, 2029

Last Updated

February 19, 2026

Status Verified

February 1, 2026

Enrollment Period

11.3 years

First QC Date

August 15, 2018

Last Update Submit

February 17, 2026

Conditions

Keywords

gene therapyOTC DeficiencyUrea Cycle Disorder

Outcome Measures

Primary Outcomes (1)

  • Number of Participants with Adverse Events and Serious Adverse Events

    Up to 416 weeks

Secondary Outcomes (2)

  • Change from Baseline Over Time in the Ureagenesis Rate

    Baseline (average of Screening and Day 1) up to 416 weeks following DTX301 administration

  • Change from Baseline Over Time in 24-Hour Area Under the Curve for Plasma Ammonia

    Baseline (Day 0 of Study 301OTC01) up to 208 weeks following DTX301 administration

Interventions

No Intervention

Eligibility Criteria

Age18 Years+
Sexall
Age GroupsAdult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Subjects 18 years of age or older with OTC previously enrolled in Study 301OTC01

You may qualify if:

  • Completed the Week 52 visit in Study 301OTC01.
  • Willing and able to provide written informed consent.
  • Willing, able, and committed to comply with scheduled study site visits, study procedures, and requirements.

You may not qualify if:

  • Planned or current participation in another interventional clinical study that may confound the efficacy or safety evaluation of DTX301 during the duration of this study.
  • Any clinically significant medical condition that, in the opinion of the investigator, would pose a risk to subject safety or would impede the study.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (9)

The Children's Hospital Colorado

Aurora, Colorado, 80045, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

Location

Icahn School of Medicine

New York, New York, 10029, United States

Location

University Hospital Cleveland Medical Center/Case Western Reserve University

Cleveland, Ohio, 44106, United States

Location

M.A.G.I.C. Clinic

Calgary, Alberta, T2E 7Z4, Canada

Location

Hopital Femme Mere Enfant

Bron, Rhone, 69677, France

Location

Hospital Clinico Universitario de Santiago

Santiago de Compostela, Coruna, 15706, Spain

Location

Hospital Universitario de Cruces. Servicio de Pediatria

Barakaldo, Vizcaya, 48903, Spain

Location

Queen Elizabeth Hospital, Department of Endocrinology

Birmingham, B15 2TH, United Kingdom

Location

Related Links

Biospecimen

Retention: SAMPLES WITH DNA

Blood

MeSH Terms

Conditions

Ornithine Carbamoyltransferase Deficiency DiseaseUrea Cycle Disorders, Inborn

Condition Hierarchy (Ancestors)

Brain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesGenetic Diseases, X-LinkedGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesAmino Acid Metabolism, Inborn ErrorsMetabolism, Inborn ErrorsMetabolic DiseasesNutritional and Metabolic Diseases

Study Officials

  • Medical Director

    Ultragenyx Pharmaceuticals Inc

    STUDY DIRECTOR

Study Design

Study Type
observational
Observational Model
OTHER
Time Perspective
PROSPECTIVE
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

August 15, 2018

First Posted

August 17, 2018

Study Start

August 30, 2018

Primary Completion (Estimated)

December 1, 2029

Study Completion (Estimated)

December 1, 2029

Last Updated

February 19, 2026

Record last verified: 2026-02

Locations