Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis

Hereditary transthyretin amyloidosis is an autosomal dominant form of amyloidosis caused by an abnormality in transthyretin, with various ocular manifestations. Among these, ocular amyloid angiopathy has attracted attention because of its direct link to visual impairment and its correlation with sys...

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Published in:PloS one 2022-09, Vol.17 (9), p.e0275180-e0275180
Main Authors: Kakihara, Shinji, Hirano, Takao, Kitahara, Junya, Matsuda, Yorishige, Imai, Akira, Miyahara, Teruyoshi, Murata, Toshinori
Format: Article
Language:English
Subjects:
Alzheimer's disease
Amyloid
Amyloidosis
Angiography
Automation
Biology and Life Sciences
Biomarkers
Circularity
Coherence (Optics)
Correlation
Correlation analysis
Diagnosis
Evaluation
Eye (anatomy)
Glaucoma
Medical imaging
Medicine and Health Sciences
Opacity
Optical Coherence Tomography
Parameters
Perfusion
Physical Sciences
Research and Analysis Methods
Retina
Tomography
Transthyretin
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creator Kakihara, Shinji
Hirano, Takao
Kitahara, Junya
Matsuda, Yorishige
Imai, Akira
Miyahara, Teruyoshi
Murata, Toshinori
description Hereditary transthyretin amyloidosis is an autosomal dominant form of amyloidosis caused by an abnormality in transthyretin, with various ocular manifestations. Among these, ocular amyloid angiopathy has attracted attention because of its direct link to visual impairment and its correlation with systemic severity. We hypothesized that optical coherence tomography angiographic parameters would be useful biomarkers of amyloidosis systemic severity and investigated their correlation with the systemic severity score. The primary outcome was the correlation between the systemic severity score and choriocapillaris flow deficit percentage. Secondary outcomes were the correlations between the systemic severity score and retinal optical coherence tomography angiographic parameters, including foveal avascular zone size and circularity and superficial/deep/total retinal perfusion and vessel densities. The choroidal and retinal vasculature was quantified in 36 eyes from 36 patients (age, 51.8±12.1 years; disease duration, 13.4±6.2 years). Ten eyes had a history of vitrectomy for vitreous opacity. Choriocapillaris flow deficit percentage was not significantly correlated with the systemic severity score (Spearman's rank correlation: r = 2.96x10.sup.-2, p = 0.863). Similarly, foveal avascular zone size and circularity, and superficial/deep/total retinal perfusion and vessel densities were not significantly correlated with the systemic severity score. These results may indicate that optical coherence tomography angiographic parameters are not sufficient to predict amyloidosis severity.
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subjects Alzheimer's disease
Amyloid
Amyloidosis
Angiography
Automation
Biology and Life Sciences
Biomarkers
Circularity
Coherence (Optics)
Correlation
Correlation analysis
Diagnosis
Evaluation
Eye (anatomy)
Glaucoma
Medical imaging
Medicine and Health Sciences
Opacity
Optical Coherence Tomography
Parameters
Perfusion
Physical Sciences
Research and Analysis Methods
Retina
Tomography
Transthyretin
title Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis
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