Cargando…
Idiopathic Systemic Capillary Leak Syndrome
Idiopathic systemic capillary leak syndrome (ISCLS) is a very rare life-threatening disorder characterized by recurrent episodes of hypotension, hemoconcentration, and hypoalbuminemia. It is caused by transient endothelial dysfunction, leading to plasma leakage from intravascular to interstitial spa...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971649/ https://www.ncbi.nlm.nih.gov/pubmed/29910550 http://dx.doi.org/10.4103/ijccm.IJCCM_464_17 |
_version_ | 1783326309587353600 |
---|---|
author | Hajare, Kashmira Rajendra Patil, Priya Bansode, Jyoti |
author_facet | Hajare, Kashmira Rajendra Patil, Priya Bansode, Jyoti |
author_sort | Hajare, Kashmira Rajendra |
collection | PubMed |
description | Idiopathic systemic capillary leak syndrome (ISCLS) is a very rare life-threatening disorder characterized by recurrent episodes of hypotension, hemoconcentration, and hypoalbuminemia. It is caused by transient endothelial dysfunction, leading to plasma leakage from intravascular to interstitial space. Here, we report a case of ISCLS with recurrent episodes of capillary leak which required a long-term prophylaxis with beta-2 adrenergic receptor agonist and theophylline. Although ISCLS is the rare entity, associated morbidity and mortality require physician's awareness to provide timely therapy. Under-recognition in the medical community and rarity of this syndrome has precluded analysis in rational clinical trial designs that are necessary to determine targeted and adequate therapy. This report is meant to enhance awareness of ISCLS among physician's community. |
format | Online Article Text |
id | pubmed-5971649 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-59716492018-06-15 Idiopathic Systemic Capillary Leak Syndrome Hajare, Kashmira Rajendra Patil, Priya Bansode, Jyoti Indian J Crit Care Med Case Report Idiopathic systemic capillary leak syndrome (ISCLS) is a very rare life-threatening disorder characterized by recurrent episodes of hypotension, hemoconcentration, and hypoalbuminemia. It is caused by transient endothelial dysfunction, leading to plasma leakage from intravascular to interstitial space. Here, we report a case of ISCLS with recurrent episodes of capillary leak which required a long-term prophylaxis with beta-2 adrenergic receptor agonist and theophylline. Although ISCLS is the rare entity, associated morbidity and mortality require physician's awareness to provide timely therapy. Under-recognition in the medical community and rarity of this syndrome has precluded analysis in rational clinical trial designs that are necessary to determine targeted and adequate therapy. This report is meant to enhance awareness of ISCLS among physician's community. Medknow Publications & Media Pvt Ltd 2018-05 /pmc/articles/PMC5971649/ /pubmed/29910550 http://dx.doi.org/10.4103/ijccm.IJCCM_464_17 Text en Copyright: © 2018 Indian Journal of Critical Care Medicine http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Hajare, Kashmira Rajendra Patil, Priya Bansode, Jyoti Idiopathic Systemic Capillary Leak Syndrome |
title | Idiopathic Systemic Capillary Leak Syndrome |
title_full | Idiopathic Systemic Capillary Leak Syndrome |
title_fullStr | Idiopathic Systemic Capillary Leak Syndrome |
title_full_unstemmed | Idiopathic Systemic Capillary Leak Syndrome |
title_short | Idiopathic Systemic Capillary Leak Syndrome |
title_sort | idiopathic systemic capillary leak syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971649/ https://www.ncbi.nlm.nih.gov/pubmed/29910550 http://dx.doi.org/10.4103/ijccm.IJCCM_464_17 |
work_keys_str_mv | AT hajarekashmirarajendra idiopathicsystemiccapillaryleaksyndrome AT patilpriya idiopathicsystemiccapillaryleaksyndrome AT bansodejyoti idiopathicsystemiccapillaryleaksyndrome |