Table 2.
Demographic characteristics and sex and mutation status of Taiwanese patients in FOS with cardiac symptoms and IVS4 or classical Fabry mutations (n = 59)
| Characteristic | N = 59 |
|---|---|
| Median (range) age at extract, years | 60.7 (15.0–86.9) |
| Aged <18 years at extract, n (%) | 2 (3) |
| Male, n (%) | 36 (61) |
| Female, n (%) | 23 (39) |
| Residual α-Gal A activity, % of midpoint of normal range, mean (SD) | |
| IVS4 overall (n = 41) | 21.6 (21.0) |
| IVS4 male (n = 30) | 10.4 (4.5) |
| IVS4 female (n = 11) | 52.2 (17.4) |
| Classical Fabry overall (n = 16)a | 31.4 (25.0) |
| Classical male (n = 4)a | 2.9 (3.5) |
| Classical female (n = 12) | 41.0 (21.2) |
| Sex and Fabry mutation status, n (%) | |
| IVS4 overall | 41 (69)b |
| IVS4 male | 30 (73) |
| IVS4 female | 11 (27) |
| Classical overall | 18 (31)b |
| Classical male | 6 (33) |
| Classical female | 12 (67) |
a n = 2 missing
bPrevalence, IVS4 overall vs. classical overall; p < 0.05