IN VIVO OBSERVATION OF RETINAL VASCULAR DEPOSITS USING ADAPTIVE OPTICS IMAGING IN FABRY DISEASE.
Journal
Retina (Philadelphia, Pa.)
ISSN: 1539-2864
Titre abrégé: Retina
Pays: United States
ID NLM: 8309919
Informations de publication
Date de publication:
Aug 2020
Aug 2020
Historique:
pubmed:
1
10
2019
medline:
22
6
2021
entrez:
1
10
2019
Statut:
ppublish
Résumé
To report a novel finding in patients with Fabry disease, that is, the observation by adaptive optics ophthalmoscopy of intracellular lipidic deposits in retinal vessels. Observational two-center case series. Eighteen patients with genetically proven Fabry disease underwent flood-illumination adaptive optics ophthalmoscopy imaging (rtx1; Imagine Eyes, Orsay, France) of retinal vessels. Fourteen patients (78% of all patients; 7 of the 10 women and 7 of the 8 men) showed paravascular punctuate or linear opacities in both eyes. In the least-affected patients, these were seen only in the wall of precapillary arterioles as discrete spots of 5 µm to 10 µm large, whereas in those more severely affected, capillaries and first-order vessels were also involved with diffuse opacification of the wall. These deposits sometime showed a striated pattern, suggesting colocalization with vascular smooth muscle cells. Adaptive optics ophthalmoscopy of retinal vessels may be of interest for patients with Fabry disease, providing noninvasive, gradable evaluation of microvascular involvement.
Identifiants
pubmed: 31568064
doi: 10.1097/IAE.0000000000002648
pii: 00006982-202008000-00020
doi:
Substances chimiques
Sphingolipids
0
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
1623-1629Références
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