Table 2 Baseline characteristics of patients on ERT with more than 6 months of follow-up (see Figure 1 A)
From: Long term enzyme replacement therapy for Fabry disease: effectiveness on kidney, heart and brain
Baseline (prior to ERT) | Males | Females | Adolescents |
|---|---|---|---|
| Â | ERT | ERT | ERT |
N | 30 | 27 | 6 (2 males) |
Agalsidase alfa | 9 | 10 | 4 |
Agalsidase beta | 21 | 17 | 2 |
Age start ERT, mean ± SD and median (range) | 38.9 ± 14.3 | 46.8 ± 12.3 | 16.6 ± 0.7 |
| Â | 40.2 (18.0-65.3) | 47.2 (20.8-71.5) | 16.6 (15.9-17.7) |
ACE-ARB (%) | 8 (26.7) | 10 (37.0) | 0 (0) |
Hypertension | 5 (16.7) | 8 (29.6) | 0 (0) |
eGFR (ml/min/1.73 m2) | 88.5 ± 40.6 | 86.6 ± 31.3 | 150.4 ± 42.8 |
CKD 1-5 (%) | 23 (76.7) | 21 (77.8) | 3 (50) |
LVH (%) | 11/27 (40.7) | 12/24 (50.0) | 0/4 (0) |
WML (%) | 12/22 (54.5) | 21/27 (77.8) | 2/6 (33.3) |
Dialysis (%) | 0 (0) | 0 (0) | 0 (0) |
Kidney transplant (%) | 0 (0) | 1 (3.7) | 0 (0) |
Cardiac complication (%) | 4 (13.3) | 1 (3.7) | 0 (0) |
Stroke (%) | 2 (6.7) | 1 (3.7) | 0 (0) |