Dutch cohort.
| Group (age median, range) | Median age (range) | n | Percentage CV (n, 95% CI) | Details |
|---|---|---|---|---|
| Classical | ||||
| Males | 40 (19–65) | 35 | 94% (33, 80- 99) | 2 subjects without CV received >9 years of treatment with ERT |
| Females | 48 (19–81) | 66 | 82% (54, 71–89 ) | 5 subjects with CV received amiodarone treatment before or during CV assessment |
| Total | 101 | 86% (87, 78–92) | ||
| Non-classical | ||||
| Males | 64 (36–74) | 11 | 27% (3, 9–57) | In all patients with CV, corneal changes were minimal |
| Females | 39 (18–78) | 14 | 7% (1, <0.01–34) | |
| Total | 25 | 16% (4, 6–35) | ||
| Uncertain | ||||
| Males | 49 (45–71) | 5 | 0 (0–48) | |
| Females | 57 (30–68) | 6 | 0 (0–44) | |
| Total | 11 | 0 (0–30) | ||
| No FD | ||||
| Males | 41 (23–70) | 5 | 0 (0–48) | |
| Females | 49 (52–46) | 2 | 0 (0–71) | |
| Total | 7 | 0 (0–40) |
CV, cornea verticillata, ERT, enzyme replacement therapy. FD, Fabry disease.