Table 1 Patients’ characteristics and medical history
Demographics | n (md) | Mean (SD) |
|---|---|---|
Age at inclusion (years) | 17 (1) | 41.5 (11.3) |
Height (cm) | 17 (1) | 176.7 (6.5) |
Weight (kg) | 17 (1) | 72.8 (13.1) |
Heart rate (bpm) | 15 (3) | 67.1 (14.8) |
Systolic blood pressure (mm Hg) | 15 (3) | 123.0 (15.9) |
Diastolic blood pressure (mm Hg) | 15 (3) | 77.6 (13.7) |
Medical history | n (md) | n (%) |
Angiokeratomas | 17 (1) | 13 (76.5) |
Kidney failure | 17 (1) | 7 (41.2) |
Dialysis | 7 | 4 (57.1) |
Kidney transplantation | 7 | 3 (42.9) |
Coronary insufficiency | 17 (1) | 1 (5.9) |
Hypertrophic cardiomyopathy | 17 (1) | 9 (52.9) |
Replacement therapy | 17 (1) | 17 (100) |
Duration of replacement therapy (years), mean (SD) | 17 (1) | 4.8 ± 4.6 |
Active compound | 17 (1) | |
α-galactosidase | 10 (58.8) | |
β-galactosidase | 7 (41.2) |