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Table 5 Summary of monitoring assessments to be made during ERT

From: Diagnosis and treatment of late-onset Pompe disease in the Middle East and North Africa region: consensus recommendations from an expert group

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

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