|
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)
|
- Abbreviations: SD = Standard deviation; md = Missing data
