Table 1.
Evolution of plasma GL-3 levels, anti-agalsidase beta antibodies titer and echocardiographic parameters while on ERT with agalsidase beta
| Time on enzyme replacement therapy | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Baseline | 1 month | 2 months | 3 months | 6 months | 9 months | 12 months | 24 months | 30 months | |
| Plasma GL-3a | 6.6 | 6.3 | 4.3 | 4.4 | 6.4 | – | 6.5 | 5.7 | – |
| IgG antibodiesb | – | – | – | – | (+) | – | 1/400 | 1/200 | – |
| Echocardiographic parameters | |||||||||
| LVIDD | 48 | – | – | – | – | 50 | – | 54 | 58 |
| PWTD | 10 | – | – | – | – | 11 | – | 11 | 10 |
| IVSTD | 12 | – | – | – | – | 13 | – | 8 | 7 |
| LVMI | 69 | – | – | – | – | 83 | – | 68 | 67 |
Blood samples for GL-3 assay in plasma and for anti-agalsidase antibodies detection in serum were always collected at the start of the corresponding hemodialysis session
aGL-3 plasma levels, normal range: 1.6–3.3 μmol/l (Laboratory Medicine / Clinical Chemistry, Sahlgren University Hospital, Molndal, Sweden)
bAnti-agalsidase beta IgG antibodies (Genzyme Corporation, Cambridge, MA 02142, USA)
Echocardiographic dimensions (mm): LVIDD Left Ventricular Internal Diameter in Diastole, PWTD Posterior Wall Thickness in Diastole, IVSTD Interventricular Septum Thickness in Diastole, LVMI Left Ventricular Mass Index, expressed as g/m^2.7