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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
  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
  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)
  1. LVH: left ventricular hypertrophy. WML: white matter lesions, CKD 1-5: chronic kidney disease stage 1-5. The presence of LVH or cerebral white matter lesions was not known for all patients at baseline.