Table 5.

Summary of monitoring assessments to be made during ERT

Test	Frequency
Manual muscle testing
Quantitative muscle testing
Vital capacity	Every 6 months
Time on ventilation daily (invasive or noninvasive)
Six-minute walk test
Quality of life measurement
Creatine kinase	Every 3 months in the first year post-diagnosis
Alanine transaminase
Aspartate transaminase	Every 6 months if stable on ERT
Antibody titers
Electrocardiography	At baseline and repeated regularly if clinically indicated
Echocardiography
DEXA (bone mineral density)
Audiology assessment

DEXA dual-energy X-ray absorptiometry, ERT enzyme replacement therapy