Comparison of structural progression between ciliopathy and non-ciliopathy associated with autosomal recessive retinitis pigmentosa.
Orphanet J Rare Dis
; 14(1): 187, 2019 08 01.
Article
em En
| MEDLINE
| ID: mdl-31370859
ABSTRACT
BACKGROUND:
To evaluate and compare the progression of ciliopathy and non-ciliopathy autosomal recessive Retinitis Pigmentosa patients (arRP) by measuring the constriction of hyperautofluorescent rings in fundus autofluorescence (FAF) images and the progressive shortening of the ellipsoid zone line width obtained by spectral-domain optical coherence tomography (SD-OCT).RESULTS:
For the ciliopathy group, the estimated mean shortening of the ellipsoid zone line was 259 µm per year and the ring area decreased at a rate of 2.46 mm2 per year. For the non-ciliopathy group, the estimated mean shortening of the ellipsoid zone line was 84 µm per year and the ring area decreased at a rate of 0.7 mm2 per year.CONCLUSIONS:
Our study was able to quantify and compare the loss of EZ line width and short-wavelength autofluorescence (SW-AF) ring constriction progression over time for ciliopathy and non-ciliopathy arRP genes. These results may serve as a basis for modeling RP disease progression, and furthermore, they could potentially be used as endpoints in clinical trials seeking to promote cone and rod survival in RP patients.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Retinose Pigmentar
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Ciliopatias
Tipo de estudo:
Risk_factors_studies
Limite:
Adolescent
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Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Orphanet J Rare Dis
Assunto da revista:
MEDICINA
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Estados Unidos