Figure 3. Dosing profile of recombinant human acid alpha-glucosidase (rhGAA) and immunomodulation, as well as urinary glucose tetrasaccharide (mmol/mol creatinine) over time for Patient 3.

Glc₄, depicts glucose tetrasaccharide; ERT, enzyme replacement therapy; IVIG, i.v. immunoglobulin. Upper limit of normal urinary Glc₄ for age < 6 months: 19 mmol/mol creatinine; age 6–12 months: 14 mmol/mol creatinine; age ≥ 1 year: 4.4 mmol/mol of creatinine (19).