Abstract
Purpose :
Fabry disease is a rare X-linked lysosomal storage disease. Fabry disease causes renal failure, cardiac dysfunction, cerebrovascular disease, gastrointestinal symptoms, chronic pain, and ocular abnormalities, thus negatively impacting the quality of life. In this study, we evaluated age- and gender-related anterior segment ocular manifestations in a rat model of Fabry disease.
Methods :
The study was approved by the institutional animal care and use committee and followed ARVO guidelines. A Dark Agouti rat model, α-galactosidase A (Gla-m2) knock out was generated at the Medical College of Wisconsin’s Transgenic Core facility. Rats were divided into three age groups - Group I: 8-24 weeks, Group II: 25-60 weeks, and Group III: 61+ weeks. We clinically examined intraocular pressure (IOP) using an iCare TONOLAB tonometer, corneal surface using a Topcon Slit Lamp Microscope, and the corneal thickness and anterior chamber depth (ACD) using an Optical Coherence Tomography (OCT) Bioptigen 2200 Imaging Platform. Intraocular pressure was evaluated by taking five measurements per eye and averaging the left and right eye values. The corneal surface and lens opacity were scored to determine the presence and severity of honey-comb patterns and deposits of the lens, respectively.
Results :
Clinical eye evaluations in our Gla-m2 rat model exhibited significant differences in corneal scores, lens score, ACD, corneal thickness, and alterations in IOP based on age and gender.
Conclusions :
In conclusion, we found significant alterations in ocular parameters clinically evaluated based on age and gender in a rat model of Fabry disease.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.