Table 1 Patient demographics and clinical characteristics
From: Paediatric Fabry disease: prognostic significance of ocular changes for disease severity
Variable | Patients | ||
|---|---|---|---|
Boys | Girls | Total | |
All FOS paediatric patients with ocular exam, n (%) | 131 (56.5) | 101 (43.5) | 232 (100) |
Children with any eye finding, n (%) | 62/131 (47.3) | 55/101 (54.5) | 117/232 (50.4) |
Children with CV, n (%) | 55/131 (42.0) | 53/101 (52.5) | 108/232 (46.6) |
Children with tortuous vesselsa, n (%) | 32/131 (24.4) | 17/98 (17.3) | 49/229 (21.4) |
Children with posterior spoke-like lens opacity, n (%) | 2/130 (1.5) | 3/97 (3.1) | 5/227 (2.2) |
Age of patients at first eye finding, years, median (range) | 10.3 (2.3, 17.7) | 8.9 (3.2, 16.4) | 9.7 (2.3, 17.7) |
Agalsidase alfa treatment, n (%) | |||
No | 35 (26.7) | 60 (59.4) | 95 (40.9) |
Yes (any time) | 96 (73.3) | 41 (40.6) | 137 (59.1) |
Patients with agalsidase alfa treatment and any eye finding | 54 (56.3) | 27 (65.9) | 81 (59.1) |
Patients with agalsidase alfa treatment and no eye findings | 42 (43.7) | 14 (34.1) | 56 (40.9) |