The central retinal thickness and its related genotype in ABCA4-related retinopathy

Eye (Lond). 2024 Oct;38(14):2718-2723. doi: 10.1038/s41433-024-03104-2. Epub 2024 May 13.

Abstract

Purpose: To further explore the influence of genotype, including mutation type and structural domain, on the severity of macular atrophy, we measured the central retinal thickness (CRT) in patients with ABCA4-related retinopathy.

Methods: A total of 66 patients were included in the cohort. This was a retrospective investigation. The patients were tested using whole exon sequencing and ophthalmic exams, including slip lamp exams, best-corrected visual acuity, optical coherence tomography, fundus photo, and fundus autofluorescence.

Results: In this study, we discovered that mutations on nucleotide binding domains (NBD) lead to less CRT (45.00 ± 25.25μm, 95% CI: 31.54-58.46) had significantly less CRT than the others (89.75 ± 71.17μm, 95% CI: 30.25-149.25, p = 0.032), and could accelerate the rate of CRT decrease.

Conclusions: Our study provides new perspectives in the understanding of ABCA4-related retinopathy.

MeSH terms

  • ATP-Binding Cassette Transporters* / genetics
  • Adolescent
  • Adult
  • Child
  • DNA Mutational Analysis
  • Female
  • Fluorescein Angiography / methods
  • Genotype*
  • Humans
  • Male
  • Middle Aged
  • Mutation*
  • Retina* / diagnostic imaging
  • Retina* / pathology
  • Retrospective Studies
  • Tomography, Optical Coherence* / methods
  • Visual Acuity* / physiology
  • Young Adult

Substances

  • ABCA4 protein, human
  • ATP-Binding Cassette Transporters