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Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease of the fibrovascular tissue resulting in visceral vascular malformations and (muco-) cutaneous telangiectases with recurrent bleedings. The mechanism behind the disease is not fully understood; however, observations from HH...

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Autores principales: Geisthoff, Urban, Nguyen, Ha-Long, Lefering, Rolf, Maune, Steffen, Thangavelu, Kruthika, Droege, Freya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290907/
https://www.ncbi.nlm.nih.gov/pubmed/32429545
http://dx.doi.org/10.3390/jcm9051507
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author Geisthoff, Urban
Nguyen, Ha-Long
Lefering, Rolf
Maune, Steffen
Thangavelu, Kruthika
Droege, Freya
author_facet Geisthoff, Urban
Nguyen, Ha-Long
Lefering, Rolf
Maune, Steffen
Thangavelu, Kruthika
Droege, Freya
author_sort Geisthoff, Urban
collection PubMed
description Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease of the fibrovascular tissue resulting in visceral vascular malformations and (muco-) cutaneous telangiectases with recurrent bleedings. The mechanism behind the disease is not fully understood; however, observations from HHT mouse models suggest that mechanical trauma may induce the formation of abnormal vessels. To assess the influence of environmental trauma (mechanical or light induced) on the number of telangiectases in patients with HHT, the number of telangiectases on the hands, face, and lips were counted on 103 HHT patients possessing at least three out of four Curaçao criteria. They were then surveyed for information concerning their dominant hand, exposure to sunlight, and types of regular manual work. Patients developed more telangiectases on their dominant hand and lower lip (Wilcoxon rank sum test: p < 0.001). Mechanical stress induced by manual work led to an increased number of telangiectases on patients’ hands (Mann–Whitney U test: p < 0.001). There was also a positive correlation between sun exposure and the number of telangiectases on the lips (Mann–Whitney U test: 0.027). This study shows that mechanical and UV-induced trauma strongly influence the formation of telangiectases in HHT patients. This result has potential implications in preventive measures and on therapeutic approaches for HHT.
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spelling pubmed-72909072020-06-17 Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia Geisthoff, Urban Nguyen, Ha-Long Lefering, Rolf Maune, Steffen Thangavelu, Kruthika Droege, Freya J Clin Med Article Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease of the fibrovascular tissue resulting in visceral vascular malformations and (muco-) cutaneous telangiectases with recurrent bleedings. The mechanism behind the disease is not fully understood; however, observations from HHT mouse models suggest that mechanical trauma may induce the formation of abnormal vessels. To assess the influence of environmental trauma (mechanical or light induced) on the number of telangiectases in patients with HHT, the number of telangiectases on the hands, face, and lips were counted on 103 HHT patients possessing at least three out of four Curaçao criteria. They were then surveyed for information concerning their dominant hand, exposure to sunlight, and types of regular manual work. Patients developed more telangiectases on their dominant hand and lower lip (Wilcoxon rank sum test: p < 0.001). Mechanical stress induced by manual work led to an increased number of telangiectases on patients’ hands (Mann–Whitney U test: p < 0.001). There was also a positive correlation between sun exposure and the number of telangiectases on the lips (Mann–Whitney U test: 0.027). This study shows that mechanical and UV-induced trauma strongly influence the formation of telangiectases in HHT patients. This result has potential implications in preventive measures and on therapeutic approaches for HHT. MDPI 2020-05-17 /pmc/articles/PMC7290907/ /pubmed/32429545 http://dx.doi.org/10.3390/jcm9051507 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
Geisthoff, Urban
Nguyen, Ha-Long
Lefering, Rolf
Maune, Steffen
Thangavelu, Kruthika
Droege, Freya
Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title_full Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title_fullStr Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title_full_unstemmed Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title_short Trauma Can Induce Telangiectases in Hereditary Hemorrhagic Telangiectasia
title_sort trauma can induce telangiectases in hereditary hemorrhagic telangiectasia
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290907/
https://www.ncbi.nlm.nih.gov/pubmed/32429545
http://dx.doi.org/10.3390/jcm9051507
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