Longitudinal Study to Assess the Quantitative Use of Fundus Autofluorescence for Monitoring Disease Progression in Choroideremia.

ABSTRACT:

Background

Characterisation of preserved autofluorescence (PAF) area in choroideremia (CHM) and its validity for monitoring disease progression in clinical trials is of importance.

Methods

Eighty patients with molecularly confirmed CHM were recruited. PAF area was measured manually by 2 graders and half-life was calculated based on exponential decay model.

Results

Mean age at baseline and follow-up examination was 38.1 (range, 10-69) and 40.7 (range, 11-70) years. Mean follow-up interval was 29 months (range, 6-104). The median LogMAR visual acuity was 0.10 (OD) and 0.18 (OS). Interobserver repeatability for PAF area was -0.99 to 1.03 mm² (-6.46 to 6.49% of area). There was a statistically significant relationship between age and rate of PAF area loss (r

SUBMITTER: Dubis AM 

PROVIDER: S-EPMC7826764 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature