Status:

UNKNOWN

Exercise Capacity in Response to Enzyme Replacement Therapy in Pediatric Pompe Disease.

Lead Sponsor:

Rambam Health Care Campus

Conditions:

Pompe Disease Infantile-Onset

Eligibility:

All Genders

5-18 years

Brief Summary

Our aims are to investigate the acute and long term effect of ERT on exercise capacity; comparing the effect of different ERT dosages (as prescribed by the clinician according to clinical judgment) an...

Detailed Description

Pompe disease is a rare autosomal recessive metabolic myopathy caused by reduced or absence activity of the lysosomal enzyme acid alpha-glucosidase (GAA). Enzyme replacement therapy (ERT) with Myozyme...

Eligibility Criteria

Inclusion

  • Pompe patients \>5 years that have been on alpha-glucosidase (GAA).

Exclusion

  • Oxygen saturation \> 90% in room air without ventilatory assistance.
  • Patients will be excluded if they required any invasive ventilation or if they required noninvasive ventilation while awake and upright

Key Trial Info

Start Date :

June 24 2018

Trial Type :

OBSERVATIONAL

Allocation :

ESTIMATED

End Date :

January 1 2024

Estimated Enrollment :

4 Patients enrolled

Trial Details

Trial ID

NCT04755751

Start Date

June 24 2018

End Date

January 1 2024

Last Update

February 16 2021

Active Locations (1)

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Rambam Medical Center

Haifa, Israel