Status:

ACTIVE_NOT_RECRUITING

Clinical Study for Treatment-naïve IOPD Babies to Evaluate Efficacy and Safety of ERT With Avalglucosidase Alfa

Lead Sponsor:

Sanofi

Conditions:

Glycogen Storage Disease Type II

Eligibility:

All Genders

Up to 12 years

Phase:

PHASE3

Brief Summary

This is a single group, treatment, Phase 3, open-label study to assess efficacy, safety, pharmacokinetic (PK), pharmacodynamics (PD) of avalglucosidase alfa in treatment-naïve male and female particip...

Detailed Description

Study duration may be variable by country, including at least completion of the PAP and ETP, and up to 4.08 years.

Eligibility Criteria

Inclusion

  • Participants must have confirmed diagnosis of infantile-onset Pompe disease defined as: the presence of 2 lysosomal acid α-glucosidase (GAA) pathogenic variants and a documented GAA deficiency from blood, skin, or muscle tissue; or the presence of 1 GAA pathogenic variant and a documented GAA deficiency from blood, skin and muscle tissue in 2 separate samples (from either 2 different tissues or from the same tissue but at 2 different sampling dates).
  • Participants must have established cross-reactive immunological material (CRIM) status available prior to enrollment.
  • Participants must have cardiomyopathy at the time of diagnosis: ie, left ventricular mass index (LVMI) equivalent to mean age specific LVMI
  • +1 standard deviation for participants diagnosed by newborn screening or sibling screening;
  • +2 standard deviation for participants diagnosed by clinical evaluation.
  • Parents or legally authorized representative(s) must be capable of giving signed informed consent.

Exclusion

  • Participants with symptoms of respiratory insufficiency, including any ventilation use (invasive or noninvasive) at the time of enrollment.
  • Participants with major congenital abnormality.
  • Participants with clinically significant organic disease (with the exception of symptoms relating to Pompe disease).
  • Participant received any Pompe disease specific treatment, eg enzyme-replacement gene therapy (ERT).
  • Participant who has previously been treated in any clinical trial of avalglucosidase alfa.
  • Participant not suitable for participation, whatever the reason, as judged by the Investigator, including medical or clinical conditions, or participants potentially at risk of noncompliance to study procedures.
  • The above information is not intended to contain all considerations relevant to a patient's potential participation in a clinical trial.

Key Trial Info

Start Date :

September 1 2021

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

August 10 2027

Estimated Enrollment :

17 Patients enrolled

Trial Details

Trial ID

NCT04910776

Start Date

September 1 2021

End Date

August 10 2027

Last Update

March 13 2025

Active Locations (16)

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Page 1 of 4 (16 locations)

1

Stanford Hospital- Site Number : 8400006

Stanford, California, United States, 94305

2

Children's Hospitals and Clinics of Minnesota- Site Number : 8400008

Minneapolis, Minnesota, United States, 55404

3

Advanced Medical Genetics- Site Number : 8400002

Hawthorne, New York, United States, 10532

4

Duke University Medical Center- Site Number : 8400004

Durham, North Carolina, United States, 27710