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Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study
Evidence supports that hereditary angioedema (HAE) may be considered as a paroxysmal permeability disorder with defective but self-limiting endothelial barrier dysfunction. A potential subclinical abnormal vascular permeability at retinal capillaries could induce damage resulting in retinopathy. We...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6969431/ https://www.ncbi.nlm.nih.gov/pubmed/31952522 http://dx.doi.org/10.1186/s13023-019-1263-6 |
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author | Triggianese, Paola Cesareo, Massimo Guarino, Maria Domenica Conigliaro, Paola Chimenti, Maria Sole Cedola, Francesca Mazzeo, Caterina Nucci, Carlo Perricone, Roberto |
author_facet | Triggianese, Paola Cesareo, Massimo Guarino, Maria Domenica Conigliaro, Paola Chimenti, Maria Sole Cedola, Francesca Mazzeo, Caterina Nucci, Carlo Perricone, Roberto |
author_sort | Triggianese, Paola |
collection | PubMed |
description | Evidence supports that hereditary angioedema (HAE) may be considered as a paroxysmal permeability disorder with defective but self-limiting endothelial barrier dysfunction. A potential subclinical abnormal vascular permeability at retinal capillaries could induce damage resulting in retinopathy. We aimed at exploring for the first time the presence of microangiopathy at retinal level from a highly selective cohort of patients with HAE due to C1 esterase inhibitor protein (C1INH) deficiency (type I). We conducted a pilot, prospective, case-control study including 20 type I HAE patients and 20 age−/sex-matched healthy controls (HC). All participants underwent standard ophthalmological examination including visual fields. Superficial and deep capillary plexi in the retina were analyzed by using new optical coherence tomography angiography (OCT-A). A total of 40 eyes from 20 HAE patients and 20 eyes from HC were evaluated. Perimetric indices of visual field were slightly worse in HAE than in controls. OCT-angiograms documented in HAE patients a lower retinal capillary density in both superficial and deep scans and a higher retinal thickness compared to healthy eyes. Our findings firstly documented subclinical abnormalities in retinal microvascular network in type I HAE patients that might be associated with early subtle functional changes. This preliminary evidence supports the hypothesis of a recurrent endothelial barrier failure at retinal level in HAE patients potentially resulting in chronic damage. |
format | Online Article Text |
id | pubmed-6969431 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-69694312020-01-27 Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study Triggianese, Paola Cesareo, Massimo Guarino, Maria Domenica Conigliaro, Paola Chimenti, Maria Sole Cedola, Francesca Mazzeo, Caterina Nucci, Carlo Perricone, Roberto Orphanet J Rare Dis Letter to the Editor Evidence supports that hereditary angioedema (HAE) may be considered as a paroxysmal permeability disorder with defective but self-limiting endothelial barrier dysfunction. A potential subclinical abnormal vascular permeability at retinal capillaries could induce damage resulting in retinopathy. We aimed at exploring for the first time the presence of microangiopathy at retinal level from a highly selective cohort of patients with HAE due to C1 esterase inhibitor protein (C1INH) deficiency (type I). We conducted a pilot, prospective, case-control study including 20 type I HAE patients and 20 age−/sex-matched healthy controls (HC). All participants underwent standard ophthalmological examination including visual fields. Superficial and deep capillary plexi in the retina were analyzed by using new optical coherence tomography angiography (OCT-A). A total of 40 eyes from 20 HAE patients and 20 eyes from HC were evaluated. Perimetric indices of visual field were slightly worse in HAE than in controls. OCT-angiograms documented in HAE patients a lower retinal capillary density in both superficial and deep scans and a higher retinal thickness compared to healthy eyes. Our findings firstly documented subclinical abnormalities in retinal microvascular network in type I HAE patients that might be associated with early subtle functional changes. This preliminary evidence supports the hypothesis of a recurrent endothelial barrier failure at retinal level in HAE patients potentially resulting in chronic damage. BioMed Central 2020-01-17 /pmc/articles/PMC6969431/ /pubmed/31952522 http://dx.doi.org/10.1186/s13023-019-1263-6 Text en © The Author(s). 2020 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Letter to the Editor Triggianese, Paola Cesareo, Massimo Guarino, Maria Domenica Conigliaro, Paola Chimenti, Maria Sole Cedola, Francesca Mazzeo, Caterina Nucci, Carlo Perricone, Roberto Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title | Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title_full | Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title_fullStr | Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title_full_unstemmed | Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title_short | Evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
title_sort | evaluation of retinal microvascular perfusion in hereditary angioedema: a case-control study |
topic | Letter to the Editor |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6969431/ https://www.ncbi.nlm.nih.gov/pubmed/31952522 http://dx.doi.org/10.1186/s13023-019-1263-6 |
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