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The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease that involves the endothelium and microcirculation, leading to an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. The consequence of the capillary leakage is a life-threatening hypovolem...

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Autores principales: Colombo, Riccardo, Wu, Maddalena Alessandra, Catena, Emanuele, Perotti, Andrea, Fossali, Tommaso, Cioffi, Federico, Rech, Roberto, Castelli, Antonio, Cicardi, Marco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5919959/
https://www.ncbi.nlm.nih.gov/pubmed/29732372
http://dx.doi.org/10.3389/fmed.2018.00111
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author Colombo, Riccardo
Wu, Maddalena Alessandra
Catena, Emanuele
Perotti, Andrea
Fossali, Tommaso
Cioffi, Federico
Rech, Roberto
Castelli, Antonio
Cicardi, Marco
author_facet Colombo, Riccardo
Wu, Maddalena Alessandra
Catena, Emanuele
Perotti, Andrea
Fossali, Tommaso
Cioffi, Federico
Rech, Roberto
Castelli, Antonio
Cicardi, Marco
author_sort Colombo, Riccardo
collection PubMed
description Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease that involves the endothelium and microcirculation, leading to an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. The consequence of the capillary leakage is a life-threatening hypovolemic shock that can lead to lethal multiple organ dysfunction. The autonomic nervous system (ANS) is central in regulating the cardiovascular response to hypovolemia, but ANS modulation in ISCLS has not yet been investigated. Here, we report ANS activity during acute phase and recovery from a severe ISCLS shock and speculate on the possibility that autonomic mechanisms underlie the pathogenesis of attacks.
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spelling pubmed-59199592018-05-04 The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome Colombo, Riccardo Wu, Maddalena Alessandra Catena, Emanuele Perotti, Andrea Fossali, Tommaso Cioffi, Federico Rech, Roberto Castelli, Antonio Cicardi, Marco Front Med (Lausanne) Medicine Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease that involves the endothelium and microcirculation, leading to an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. The consequence of the capillary leakage is a life-threatening hypovolemic shock that can lead to lethal multiple organ dysfunction. The autonomic nervous system (ANS) is central in regulating the cardiovascular response to hypovolemia, but ANS modulation in ISCLS has not yet been investigated. Here, we report ANS activity during acute phase and recovery from a severe ISCLS shock and speculate on the possibility that autonomic mechanisms underlie the pathogenesis of attacks. Frontiers Media S.A. 2018-04-20 /pmc/articles/PMC5919959/ /pubmed/29732372 http://dx.doi.org/10.3389/fmed.2018.00111 Text en Copyright © 2018 Colombo, Wu, Catena, Perotti, Fossali, Cioffi, Rech, Castelli and Cicardi. https://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Colombo, Riccardo
Wu, Maddalena Alessandra
Catena, Emanuele
Perotti, Andrea
Fossali, Tommaso
Cioffi, Federico
Rech, Roberto
Castelli, Antonio
Cicardi, Marco
The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title_full The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title_fullStr The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title_full_unstemmed The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title_short The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome
title_sort role of failing autonomic nervous system on life-threatening idiopathic systemic capillary leak syndrome
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5919959/
https://www.ncbi.nlm.nih.gov/pubmed/29732372
http://dx.doi.org/10.3389/fmed.2018.00111
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