Table 3.
Comparison of Age-Matched Sibling Pairs' Atrophy Area and Effective Radius Growth Rates
Family No. | Genotype Class* | Patient No. | Sex | Age-Match (years) | Duration of Follow-Up (years) | Visual Acuity Change | Age-Matched Progression Rates in DAF | Inter-sibling Differences in DAF Growth | |||||||||
Baseline | Final | Area Growth Rate (mm2/year) | Radius Growth Rate (mm/year) | Area Growth Rate (mm2/year) | Radius Growth Rate (mm/year) | ||||||||||||
Right | Left | Right | Left | Right | Left | Right | Left | Right | Left | Right | Left | ||||||
F1 | B | P1 | M | 29 | 7.04 | 6 | 17 | 5 | 19 | 3.27 | 3.12 | 0.21 | 0.19 | 1.117 | 1.233 | 0.009 | 0.0004 |
P2 | M | 29 | 8.51 | 23 | 30 | 20 | 31 | 2.15 | 1.89 | 0.22 | 0.19 | ||||||
F2 | B | P3 | F | 30 | 3.67 | 35 | 35 | 34 | 35 | 1.01 | 0.30 | 0.20 | 0.07 | 0.870 | 0.147 | 0.165 | 0.042 |
P4 | M | 30 | 7.21 | 62 | 40 | 38 | 36 | 0.14 | 0.15 | 0.03 | 0.03 | ||||||
F3 | A | P5 | F | 39 | 2.67 | 15 | 38 | 12 | 31 | 0.45 | 1.73 | 0.02 | 0.06 | 0.273 | 0.442 | 0.013 | 0.031 |
P6 | M | 38 | 8.72 | 15 | 20 | 15 | 20 | 0.72 | 2.17 | 0.03 | 0.09 | ||||||
F4 | B | P7 | M | 58 | 5.14 | 9 | 9 | 10 | 7 | 3.89 | 3.19 | 0.12 | 0.10 | 0.917 | 1.075 | 0.062 | 0.074 |
P8 | M | 58 | 1.48 | 3 | 2 | 1 | 1 | 4.81 | 4.27 | 0.18 | 0.17 | ||||||
F5 | B | P9 | F | 57 | 3.66 | 86 | 86 | 80 | 82 | 2.02 | 1.34 | 0.15 | 0.23 | 0.467 | 0.006 | 0.043 | 0.008 |
P10 | M | 57 | 4.51 | 56 | 81 | −15 | 33 | 1.55 | 1.33 | 0.19 | 0.24 | ||||||
F7 | A | P13 | M | 18 | 2.53 | 42 | 63 | 31 | 35 | 0.36 | 0.04 | 0.05 | 0.01 | 0.176 | 0.205 | 0.032 | 0.018 |
P14 | F | 18 | 7.76 | 35 | 35 | 29 | 33 | 0.18 | 0.24 | 0.02 | 0.02 | ||||||
F10 | B | P19 | M | 75 | 3.55 | −30 | −30 | −30 | −30 | 1.15 | 1.52 | 0.06 | 0.07 | 1.539 | 0.813 | 0.037 | 0.023 |
P20 | F | 75 | 5.27 | −30 | −30 | −30 | −30 | 2.69 | 2.33 | 0.09 | 0.09 | ||||||
F14 | A | P28 | F | 27 | 0.51 | 5 | 10 | 10 | 11 | 1.17 | 3.12 | 0.13 | 0.32 | 0.677 | 1.011 | 0.078 | 0.111 |
P29 | M | 26 | 1.52 | 7 | 7 | 4 | 5 | 1.85 | 2.11 | 0.20 | 0.21 |
Definition: Class A, severe biallelic mutations. Class B, an allele of intermediate severity in trans with a severe or intermediate allele.
DAF, dark autofluorescence.