Characteristic | IVS4 mutation n = 25 | Classical Fabry mutations n = 12 | P-value |
---|---|---|---|
Sex, n (%) | Â | Â | Â |
 Male | 19 (76.0) | 4 (33.0) | 0.0274a |
 95% CI | 0.55-0.91 | 0.10-0.65 |  |
 Female | 6 (24.0) | 8 (67.0) |  |
Age at symptom onset, yearsc | Â | Â | Â |
 Mean (SD) | 50.3 (7.8) | 9.5 (2.0) |  |
 95% CI | 46.2-54.5 | 8.1-10.9 |  |
 Median (range) | 48.0 (38.0-65.0) | 10.0 (6.0-12.0) | <0.0001b |
Age at diagnosis, years | Â | Â | Â |
 Mean (SD) | 57.6 (6.9) | 39.8 (14.5) |  |
 95% CI | 54.7-60.4 | 30.6-49.1 |  |
 Median (range) | 59.0 (42.0-67.0) | 40.0 14.0-60.0) | 0.0001b |
Age at FOS entry, years | Â | Â | Â |
 Mean (SD) | 59.7 (6.7) | 42.4 (14.1) |  |
 95% CI | 56.9-62.5 | 33.4-51.4 |  |
 Median (range) | 60.3 (44.8-69.7) | 43.3 (17.4-61.2) | <0.0001b |
Received ERT, n | Â | Â | Â |
 Yes | 25 | 12 | NA |
 95% CI | 0.86-1.00 | 0.74-1.00 |  |
Age at treatment start, years | Â | Â | Â |
 Mean (SD) | 58.6 (6.8) | 40.9 (14.5) |  |
 95% CI | 55.8-61.5 | 31.7-50.1 |  |
 Median (range) | 59.7 (44.1-68.3) | 40.9 (14.9-61.3) | 0.0001b |
MRI after treatment initiation, n (%) | Â | Â | Â |
 Yes | 25 (100.0) | 10 (83.3) | 0.0991a |
 95% CI | 0.86-1.00 | 0.52-0.98 |  |
Age at MRI assessment, years | Â | Â | Â |
 Mean (SD) | 60.0 (6.8) | 42.6 (14.3) |  |
 95% CI | 57.2-62.8 | 33.6-51.7 |  |
 Median (range) | 60.7 (45.0-70.4) | 43.0 (18.0-61.4) | 0.0001b |