New hope for kids with pompe disease: experimental drug shows promise

NCT ID NCT03019406

First seen Jun 27, 2026 · Last updated Jun 27, 2026

Summary

This phase 2 trial tests a new enzyme replacement therapy called avalglucosidase alfa (Nexviazyme) in 22 children with infantile-onset Pompe disease who are not responding well to standard treatment. The study aims to see if the new drug is safe and can improve outcomes. Participants receive the drug through an IV infusion, and researchers monitor for side effects and immune responses.

What this could mean

Our plain-language read of the trial. This is informational only — not medical advice or a prediction.

Active substance

avalglucosidase alfa (Nexviazyme)

What this could lead to

If it works, this could offer a better treatment option for children with Pompe disease who are not doing well on current enzyme replacement therapy.

What could go wrong

This is a small, early-phase trial with only 22 participants, so results may not apply to all patients. The drug may cause side effects like infusion reactions.

Disclaimer Read more

This is a summary of the original study . Summaries may miss details or leave out important information. Before applying or accepting participation, make sure you have read and understood the full study. Curemydisease.com takes no responsibility whatsoever for anything missed, misunderstood, or acted upon as a result of our summary — we know it does not capture everything.

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Conditions

The condition(s) this trial relates to.

glycogen storage disease due to acid maltase deficiency, infantile onset glycogen storage disease II

As listed by the trial registrant

The condition terms exactly as the trial's registrant entered them.

Contacts and locations

Locations

  • Duke University Medical Center Site Number : 8400001

    Durham, North Carolina, 27710-4000, United States

  • Investigational Site Number : 1580001

    Hsinchu, 30059, Taiwan

  • Investigational Site Number : 2500001

    Tours, 37044, France

  • Investigational Site Number : 2500002

    Paris, 75019, France

  • Investigational Site Number : 2500003

    Paris, 75015, France

  • Investigational Site Number : 2500004

    Nantes, 44093, France

  • Investigational Site Number : 3920001

    Fuchu-shi, Tokyo, 183-0042, Japan

  • Investigational Site Number : 3920002

    Fuchu-shi, Tokyo, 183-8561, Japan

  • Investigational Site Number : 8260001

    London, London, City of, WC1N 3JH, United Kingdom

  • Investigational Site Number : 8260002

    Manchester, M13 9WL, United Kingdom

  • Regional Medical Genetics Center of New York Site Number : 8400002

    Valhalla, New York, 10595, United States

  • Seattle Childrens Hospital and Regional Medical Center- Site Number : 8400005

    Seattle, Washington, 98040, United States