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Glycogen Storage Disease Type Ia (GSDIa) Disease Monitoring Program

Recruiting
Conditions
Glycogen Storage Disease Type Ia
Interventions
Other: No Intervention
Registration Number
NCT06636383
Lead Sponsor
Ultragenyx Pharmaceutical Inc
Brief Summary

The main objective of this observational study is to evaluate the long-term safety and effectiveness of DTX401 for at least 10 years after DTX401 administration.

Detailed Description

The DTX401-CL401 Disease Monitoring Program (DMP) is a prospective, multicenter, long-term observational study to follow up participants with GSDIa for at least 10 years after the administration of DTX401.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
140
Inclusion Criteria
  • Patient who had:

    • DTX401 (full or partial dose) administered in a parent clinical study (Group 1) or
    • Prescribed DTX401(full or partial dose) administered in a post-marketing setting (Group 2)
  • Patient is willing and able to provide informed consent after the nature of the study has been explained, and prior to any research-related assessments or procedures. If a minor or an adult with cognitive limitations, the patient is willing and able (if possible) to provide assent and have a legally authorized representative provide informed consent after the nature of the study has been explained, and prior to any research-related assessments or procedures.

Exclusion Criteria
  • Presence of any condition that would interfere with study participation, interpretation of results or affect patient's safety in the opinion of the Investigator

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Group 1: DTX401 in Prior Clinical StudyNo InterventionPatients administered DTX401 (full or partial dose) in prior clinical study involving DTX401
Group 2: DTX401 in Post-Marketing SettingNo InterventionPatients administered prescribed DTX401(full or partial dose) in a post-marketing setting
Primary Outcome Measures
NameTimeMethod
Outcomes of Pregnancy in Patients Treated with DTX401 or Patient's Partner10 Years
Incidence of Pregnancy in Patients Treated with DTX401 or Patient's Partner10 Years
Incidence, Relationship, Severity and Seriousness of Adverse Events of Special Interest (AESIs) for Adeno-Associated Virus (AAV) Therapies10 Years
Incidence and Severity of Serious Adverse Events (SAEs) Assessed as Related to DTX401 by the Investigator10 Years
Incidence and Severity of SAEs of Infusion-Related Reactions Including Hypersensitivity (Group 2 only)1 Year
Incidence and Severity of SAEs Assessed by the Investigator as Related to Concomitant Immunomodulatory Therapies (Group 2 only)1 Year
Secondary Outcome Measures
NameTimeMethod
Change from Baseline Over Time in Daytime and Nighttime Percentage of Glucose Values During the 2 Weeks Leading Up to the Assessment VisitsBaseline, Up to 10 Years

The following ranges will be assessed: \< 54 mg/dL, \< 60 mg/dL, \< 70 mg/dL, 70-120 mg/dL, \> 120 mg/dL

Change from Baseline Over Time in Total Number of Hypoglycemic Events on Continuous Glucose Monitoring (CGM) During the 2 Weeks Leading Up to the Assessment VisitsBaseline, Up to 10 Years

The following ranges will be assessed: \< 54 mg/dL, \< 70 mg/dL

Change from Baseline Over Time in Total Duration of Hypoglycemic Events on CGM During the 2 Weeks Leading Up to the Assessment VisitsBaseline, Up to 10 Years

The following ranges will be assessed: \< 54 mg/dL, \< 70 mg/dL

Change from Baseline Over Time in Real-World Overnight Fasting ToleranceBaseline, Up to 10 Years
Change from Baseline Over Time in Metabolic and GSDIa Related Parameters: Height10 Years
Change from Baseline Over Time in Metabolic and GSDIa Related Parameters: Weight10 Years
Change from Baseline Over Time in Metabolic and GSDIa Related Parameters: Body Mass Index (BMI)10 Years
Number of Participants with Clinically Significant Laboratory Values10 Years
Frequency of Major Clinical Events (MCEs)10 Years
Frequency of MCEs from Review of Medical Records10 Years
Change from Baseline in Patient Experience Clinical InterviewBaseline, Up to 10 Years
Change from Baseline in European Quality of Life 5 Dimensions 5 Levels (EQ-5D-5L) Questionnaire ScoreBaseline, Up to 1 Year
Change from Baseline in European Quality of Life 5 Dimensions Youth (EQ-5D-Y) Questionnaire ScoreBaseline, Up to 1 Year
Change from Baseline Over Time in Annual Number of GSDIa Related Hospitalizations, Emergency Room Visits, Outpatient Visits, and Hospital DaysBaseline, Up to 10 Years
Change from Baseline Over Time in Missed Work/School Days in the Past 3 MonthsBaseline, Up to 10 Years
Change from Baseline in Work/School Productivity RatingBaseline, Up to 10 Years
Change from Baseline in EmploymentBaseline, Up to 10 Years
Percent Change Over Time from Baseline in Total Cornstarch IntakeBaseline, Up to 10 Years
Change from Baseline Over Time in Frequency of Cornstarch IntakeBaseline, Up to 10 Years
Change from Baseline Over Time in Number of Nighttime Awakenings for CornstarchBaseline, Up to 10 Years
Change from Baseline Over Time in Dependance on Continuous Overnight Tube Feeding for Exogenous Glucose DeliveryBaseline, Up to 10 Years
Change from Baseline Over Time in Daily Dietary Intake of Foods, Beverages, and Medical Foods/Formulas (Non-Cornstarch)Baseline, Up to 10 Years

Trial Locations

Locations (19)

Universitätsklinikum Hamburg Eppendorf

🇩🇪

Hamburg, Germany

Children's Hospital of Orange County

🇺🇸

Orange, California, United States

Children's Hospital Colorado

🇺🇸

Denver, Colorado, United States

University of Connecticut Health Center

🇺🇸

Hartford, Connecticut, United States

University of Michigan

🇺🇸

Ann Arbor, Michigan, United States

Duke University Medical Center

🇺🇸

Durham, North Carolina, United States

The Cleveland Clinic Foundation

🇺🇸

Cleveland, Ohio, United States

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

University of Texas Health Science Center at Houston

🇺🇸

Houston, Texas, United States

Primary Children's Hospital

🇺🇸

Salt Lake City, Utah, United States

Hospital de Clinicas de Porto Alegre (HCPA) - PPDS

🇧🇷

Porto Alegre, Rio Grande do Sul, Brazil

McGill University Health Center

🇨🇦

Montréal, Quebec, Canada

Rigshospitalet

🇩🇰

Copenhagen, Denmark

University of Naples

🇮🇹

Napoli, Campania, Italy

Istituto G Gaslini Ospedale Pediatrico IRCCS

🇮🇹

Genoa, Italy

Osaka City General Hospital

🇯🇵

Osaka, Japan

Fujita Health University Hospital

🇯🇵

Toyoake, Japan

Groningen University

🇳🇱

Groningen, Netherlands

Hospital Clínico Universitario de Santiago

🇪🇸

Santiago, Spain

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