From: Paediatric Fabry disease: prognostic significance of ocular changes for disease severity
Age-adjusted FOS-MSSI total score (removing CV), median (range) | |||
---|---|---|---|
With eye findings | Without eye findings | p-value | |
All patients with assessment before 18 y (n = 232) | |||
Any eye finding | 0.5 (−11.0, 20.7), n = 117 | −2.3 (−11.1, 18.8), n = 114 | <0.001 |
CV | 0.4 (−11.0, 20.7), n = 108 | −2.2 (−11.1, 18.8), n = 123 | <0.001 |
Tortuous vessels | 3.3 (−8.7, 20.7), n = 49 | −2.0 (−11.1, 20.5), n = 179 | <0.001 |
Posterior spoke-like lens opacity | 2.4 (−6.9, 20.5), n = 5 | −1.3 (−11.1, 20.7), n = 221 | 0.257 |
Girls (n = 101) | |||
Any eye finding | 0.9 (−9.7, 21.8), n = 55 | −2.7 (−9.6, 9.4), n = 46 | 0.002 |
CV | 0.9 (−9.7, 21.8), n = 53 | −2.7 (−9.6, 9.4), n = 48 | 0.002 |
Tortuous vessels | 2.2 (−4.6, 21.8), n = 17 | −1.8 (−9.7, 19.3), n = 81 | 0.003 |
Posterior spoke-like lens opacity | 1.1 (−5.9, 5.5), n = 3 | −0.7 (−9.7, 21.8), n = 94 | 0.763 |
Boys (n = 130) | |||
Any eye finding | 1.5 (−11.8, 19.8), n = 62 | −2.8 (−11.9, 18.0), n = 68 | <0.001 |
CV | 0.3 (−11.8, 19.8), n = 55 | −2.7 (−11.9, 18.0), n = 75 | 0.006 |
Tortuous vessels | 3.6 (−9.4, 19.1), n = 32 | −2.4 (−11.9, 19.8), n = 98 | <0.001 |
Posterior spoke-like lens opacity | 10.7 (1.7, 19.8), n = 2 | −1.5 (−11.9, 19.1), n = 127 | 0.092 |