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Investigation of OCTA Biomarkers in Fabry Disease: A Long Term Follow-Up of Macular Vessel Area Density and Foveal Avascular Zone Metrics.

Katerina HufendiekMigle LindziuteJessica KaufeldIngo VolkmannDorothee BrockmannSami HosariBettina HohbergerChristian MardinCarsten FrammeJan TodeKarsten Hufendiek
Published in: Ophthalmology and therapy (2023)
This is the first long-term follow-up OCTA study in FD to our knowledge. A decrease in VAD, pronounced in the peripheral circle and deeper layers, as well as an enlargement of the FAZ could be observed over time. These changes reflect the vascular remodelling during the course of the disease. Interestingly, the reduction of VAD was more pronounced in treated patients. This could be a result of enzyme replacement therapy and could be potentially used as a reliable biomarker for monitoring the treatment of the disease. A baseline examination of VAD and FAZ before treatment initiation is meaningful. Larger studies are needed to establish the use of VAD and FAZ as biomarkers for treatment monitoring.
Keyphrases
  • replacement therapy
  • healthcare
  • newly diagnosed
  • end stage renal disease
  • combination therapy
  • prognostic factors
  • patient reported outcomes