140 Participants Needed

DTX401 for von Gierke Disease

Recruiting at 20 trial locations
PC
HC
Overseen ByHCPs Contact: Medical Information
Age: Any Age
Sex: Any
Trial Phase: Academic
Sponsor: Ultragenyx Pharmaceutical Inc
Must be taking: DTX401
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial aims to monitor the long-term safety and effectiveness of DTX401, a gene therapy for Glycogen Storage Disease Type Ia (GSDIa), a rare genetic disorder affecting sugar storage in the body. The study will observe patients who have already received DTX401, either during a previous clinical trial or through a prescription. Individuals treated with DTX401 and willing to participate are a good fit for this trial. The researchers aim to gather information over at least 10 years to better understand the treatment's safety and effectiveness in real-world settings. As a Phase 4 trial, this study focuses on a treatment already FDA-approved and proven effective, aiming to understand its benefits for more patients.

Do I need to stop my current medications for the DTX401 trial?

The trial information does not specify whether you need to stop taking your current medications. It's best to discuss this with the study team or your doctor.

What is the safety track record for DTX401?

Research has shown that DTX401, also known as pariglasgene brecaparvovec, has been safe in previous studies. One study found that people generally tolerated the treatment well, which aligns with expectations for this type of gene therapy. Another source confirmed that the safety results from earlier trials met expected standards and matched initial studies. Participants were monitored for a year after receiving DTX401, and the treatment proved manageable. Overall, evidence suggests that DTX401 is safe to use, based on researchers' observations so far.12345

Why are researchers enthusiastic about this study treatment?

Researchers are excited about DTX401 (pariglasgene brecaparvovec) for Glycogen Storage Disease Type Ia (GSDIa) because it offers a novel gene therapy approach. Unlike the traditional management strategies, which mainly involve dietary modifications and cornstarch supplementation to manage symptoms, DTX401 targets the root cause of the disease by introducing a functional copy of the deficient gene. This innovative mechanism has the potential to provide a long-term solution rather than just symptom relief, making it a groundbreaking development in GSDIa treatment.

What is the effectiveness track record for DTX401?

This trial will evaluate DTX401, a gene therapy, in two different contexts. Group 1 includes patients who received DTX401 in a prior clinical study, while Group 2 involves patients receiving DTX401 in a post-marketing setting. Studies have shown that DTX401 can effectively help people with Glycogen Storage Disease Type Ia (GSDIa). Specifically, research indicates that after treatment with DTX401, many patients needed less daily cornstarch, which is important for managing this condition. By Week 96, most patients reported feeling better and experiencing fewer symptoms. This therapy works by providing a working gene to address the root cause of the disease. These findings suggest DTX401 could greatly improve life for those with GSDIa.13467

Who Is on the Research Team?

MD

Medical Director

Principal Investigator

Ultragenyx Pharmaceuticals Inc.

Are You a Good Fit for This Trial?

Inclusion Criteria

I understand the study and agree to participate, or I have a legal representative who does.
I have received DTX401 either in a clinical study or as a prescribed treatment.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Participants receive DTX401 in a prior clinical study or in a post-marketing setting

Follow-up

Participants are monitored for safety and effectiveness after treatment

Up to 10 years

What Are the Treatments Tested in This Trial?

Interventions

  • DTX401

How Is the Trial Designed?

2

Treatment groups

Experimental Treatment

Group I: Group 2: DTX401 in Post-Marketing SettingExperimental Treatment1 Intervention
Group II: Group 1: DTX401 in Prior Clinical StudyExperimental Treatment1 Intervention

Find a Clinic Near You

Who Is Running the Clinical Trial?

Ultragenyx Pharmaceutical Inc

Lead Sponsor

Trials
94
Recruited
104,000+

Dr. Emil D. Kakkis

Ultragenyx Pharmaceutical Inc

Chief Executive Officer since 2010

MD/PhD in Biological Chemistry from UCLA

Dr. Eric Crombez

Ultragenyx Pharmaceutical Inc

Chief Medical Officer since 2023

MD from Wayne State University School of Medicine

Citations

Safety and Efficacy of DTX401, an AAV8‐Mediated Liver‐ ...

In most participants, ALT reached peak values between Weeks 6 and 12. Study maximum ALT concentration reached 323 U/L on day 46 post‐DTX401 ...

Release details

Treatment with DTX401 resulted in a statistically significant and clinically meaningful reduction in daily cornstarch intake compared with placebo at Week 48.

NCT03517085 | Safety and Dose-Finding Study of DTX401 ...

The primary objective of the study is to determine the safety of single doses of DTX401, including the incidence of dose-limiting toxicities (DLTs) at each ...

Release details

At Week 96, 83% of the DTX401 group and 95% of the crossover group reported improvements in disease burden (+1 to +3 change) as measured by the ...

Pariglasgene Brecaparvovec – Application in Therapy and ...

Pariglasgene brecaparvovec is a gene therapy using a modified virus to deliver a functional gene to treat Glycogen Storage Disease Type Ia (GSDIa).

NCT03517085 | Safety and Dose-Finding Study of DTX401 ...

Participants enrolled in the 401GSDIA01 study will be monitored for 52 weeks following DTX401 administration. Participants in Cohorts 1, 2, ...

Health Technology Briefing April 2023

Pariglasgene brecaparvovec is in development for the treatment of patients with glycogen storage disease type 1a (GSD1a). GSD1a is an inherited ...