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Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-sodium-fluoride (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomogra...

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Autores principales: Gutierrez-Cardo, Antonio, Lillo, Eugenia, Murcia-Casas, Belén, Carrillo-Linares, Juan Luis, García-Argüello, Francisco, Sánchez-Sánchez, Purificación, Rodriguez-Morata, Alejandro, Baquero Aranda, Isabel, Sánchez-Chaparro, Miguel Ángel, García-Fernández, María, Valdivielso, Pedro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290446/
https://www.ncbi.nlm.nih.gov/pubmed/32397252
http://dx.doi.org/10.3390/jcm9051393
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author Gutierrez-Cardo, Antonio
Lillo, Eugenia
Murcia-Casas, Belén
Carrillo-Linares, Juan Luis
García-Argüello, Francisco
Sánchez-Sánchez, Purificación
Rodriguez-Morata, Alejandro
Baquero Aranda, Isabel
Sánchez-Chaparro, Miguel Ángel
García-Fernández, María
Valdivielso, Pedro
author_facet Gutierrez-Cardo, Antonio
Lillo, Eugenia
Murcia-Casas, Belén
Carrillo-Linares, Juan Luis
García-Argüello, Francisco
Sánchez-Sánchez, Purificación
Rodriguez-Morata, Alejandro
Baquero Aranda, Isabel
Sánchez-Chaparro, Miguel Ángel
García-Fernández, María
Valdivielso, Pedro
author_sort Gutierrez-Cardo, Antonio
collection PubMed
description Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-sodium-fluoride (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomography. This was an observational study including 18 patients with PXE. Vascular calcification was measured in Agatston units, and deposition in the skin and vessel walls was shown using target-to-background ratio (TBR). Severity of the disease was scored by Phenodex. We found higher vascular calcification in the popliteal, femoral, and aortic arch vessels compared to other vascular regions; however, the uptake of radiotracer was the highest in the aorta and femoral arteries. In the skin, the highest uptake was observed in the neck and the axillae. There was no significant association between 18F-NaF deposition in the arteries or skin and the global Phenodex score. In contrast, the Phenodex score was significantly associated in univariate analyses with the averaged vascular calcium score (p < 0.01). In the neck, patients with higher skin Phenodex scores exhibited higher radiotracer uptake. As a conclusion, because vascular calcification is physiological, our data suggested that the detection of cutaneous (neck) 18F-NaF deposits might serve to monitor the calcification process in the short-term for patients with PXE.
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spelling pubmed-72904462020-06-15 Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum Gutierrez-Cardo, Antonio Lillo, Eugenia Murcia-Casas, Belén Carrillo-Linares, Juan Luis García-Argüello, Francisco Sánchez-Sánchez, Purificación Rodriguez-Morata, Alejandro Baquero Aranda, Isabel Sánchez-Chaparro, Miguel Ángel García-Fernández, María Valdivielso, Pedro J Clin Med Article Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-sodium-fluoride (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomography. This was an observational study including 18 patients with PXE. Vascular calcification was measured in Agatston units, and deposition in the skin and vessel walls was shown using target-to-background ratio (TBR). Severity of the disease was scored by Phenodex. We found higher vascular calcification in the popliteal, femoral, and aortic arch vessels compared to other vascular regions; however, the uptake of radiotracer was the highest in the aorta and femoral arteries. In the skin, the highest uptake was observed in the neck and the axillae. There was no significant association between 18F-NaF deposition in the arteries or skin and the global Phenodex score. In contrast, the Phenodex score was significantly associated in univariate analyses with the averaged vascular calcium score (p < 0.01). In the neck, patients with higher skin Phenodex scores exhibited higher radiotracer uptake. As a conclusion, because vascular calcification is physiological, our data suggested that the detection of cutaneous (neck) 18F-NaF deposits might serve to monitor the calcification process in the short-term for patients with PXE. MDPI 2020-05-08 /pmc/articles/PMC7290446/ /pubmed/32397252 http://dx.doi.org/10.3390/jcm9051393 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Gutierrez-Cardo, Antonio
Lillo, Eugenia
Murcia-Casas, Belén
Carrillo-Linares, Juan Luis
García-Argüello, Francisco
Sánchez-Sánchez, Purificación
Rodriguez-Morata, Alejandro
Baquero Aranda, Isabel
Sánchez-Chaparro, Miguel Ángel
García-Fernández, María
Valdivielso, Pedro
Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_full Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_fullStr Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_full_unstemmed Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_short Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_sort skin and arterial wall deposits of 18f-naf and severity of disease in patients with pseudoxanthoma elasticum
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290446/
https://www.ncbi.nlm.nih.gov/pubmed/32397252
http://dx.doi.org/10.3390/jcm9051393
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