Figure 1. Dosing profile of recombinant human acid alpha-glucosidase (rhGAA) and immunomodulation, as well as rhGAA IgG antibody titers, left ventricular mass index (A and B; in g/m²), and urinary glucose tetrasaccharide (in mmol/mol creatinine) over time for Patient 1.

Glc₄, glucose tetrasaccharide; LVMI, left ventricular mass index; ERT, enzyme replacement therapy; IVIG, i.v. immunoglobulin. Upper limit of normal LVMI is 64 g/m² (18). 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).