Evaluation of Salbutamol as an Adjuvant Therapy for Pompe Disease

Inclusion Criteria: 1. Confirm diagnosis as Pompe disease 2. age 2 years or above 3. under at least 1 year regular recombinant human acid alpha glucosidase, and no dosage/frequency change in recent 3 months 4. Provide inform consent Exclusion Criteria: 1. chronic heart disease such as arrhythmia, cardiomyopathy, cardiac infarction 2. history of seizure 3. history of diabetes mellitus 4. history of hyperthyroidism 5. hypokalemia 6. pregnant 7. allergy to β-agonists 8. under medications including diuretics, digoxin, beta-blockers, etc