Macular Impairment in Fabry Disease: A Morpho-functional Assessment by Swept-Source OCT Angiography and Focal Electroretinography
Author(s) -
Angelo Maria Minnella,
Lucilla Barbano,
Elena Verrecchia,
Francesco Martelli,
Valeria Pagliei,
Gloria Gambini,
Giorgio Placidi,
Benedetto Falsini,
Aldo Caporossi,
Raffaele Manna
Publication year - 2019
Publication title -
investigative ophthalmology and visual science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.935
H-Index - 218
eISSN - 1552-5783
pISSN - 0146-0404
DOI - 10.1167/iovs.18-26052
Subject(s) - medicine , ophthalmology , fundus photography , electroretinography , retinal , fluorescein angiography
Fabry disease (FD) is a multiorgan X-linked condition characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, resulting in a progressive intralysosomal deposit of globotriaosylceramide. The aim of this study was to evaluate the macular ultrastructure of the vascular network using optical coherence tomography angiography (OCTA) and to evaluate macular function using focal electroretinography (fERG) in Fabry patients (FPs).
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