Table 2 Age-related changes and progression rates of quantitative clinical measures in patients with Bietti Crystalline Distrophy in this cohort.

From: Longitudinal structure-function analysis of molecularly-confirmed CYP4V2 Bietti Crystalline Dystrophy

Measure

Cross sectional analysis

Longitudinal analysis

Estimate (95% CI)

p-value

R2

Adj R2

Estimate (95% CI)

p-value

Marg R2

Cond R2

EZ width (µm/year) NCross: 14; NLong: 9

43.20 (−56.7 to 143.1)

0.36

0.068

−0.01

−90.35 (−134.6 to −46.09)

0.0003

0.216

0.947

Proportion-preserved EZ (%/year) NCross: 14; NLong: 9

−0.47 (−2.36 to 1.41)

0.59

0.024

−0.06

−3.31 (−6.06 to −0.56)

< 0.0001

0.234

0.995

CRT (µm/year) NCross: 22; NLong: 15

−3.01 (−5.06 to −0.96)

0.006

0.319

0.285

−2.04 (−3.73 to −0.35)

0.021

0.089

0.992

PR + RPE (µm/year) NCross: 22; NLong: 15

−1.35 (−2.24 to −0.45)

0.005

0.328

0.295

−1.15 (−1.73 to −0.58)

0.0002

0.218

0.923

Choroid (µm/year) NCross: 18; NLong: 13

−2.94 (−5.65 to −0.23)

0.036

0.248

0.201

−5.40 (−8.15 to −2.65)

< 0.0001

0.427

0.992

BCVA (LogMAR/year) NCross: 25; NLong: 23

0.007(−0.004 to 0.018)

0.19

0.074

0.034

0.016(0.006 to 0.027)

0.0019

0.252

0.790

  1. Statistically significant age-related changes (p < 0.05) are reported in bold.
  2. EZ Ellipsoid zone, CRT Central retinal thickness, PR + RPE Photoreceptor and retinal pigment epithelium complex, BCVA Best corrected visual acuity. NCross and NLong: number of patients with usable data for cross-sectional and for longitudinal analysis, respectively. Adj R2 Adjusted R2, Marg R2 Marginal R2, Cond R2 Conditional R2.
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