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VOLUME 22 , ISSUE 5 ( 2018 ) > List of Articles

CASE REPORT

Idiopathic systemic capillary leak syndrome

Priya Patil, Kashmira Hajare, Jyoti Bansode

Keywords : Agonist, beta-2 receptor, endothelial dysfunction, hemoconcentration, hypoalbuminemia, hypotension, idiopathic systemic capillary leak syndrome, theophylline

Citation Information : Patil P, Hajare K, Bansode J. Idiopathic systemic capillary leak syndrome. Indian J Crit Care Med 2018; 22 (5):369-371.

DOI: 10.4103/ijccm.IJCCM_464_17

License: CC BY-ND 3.0

Published Online: 01-05-2018

Copyright Statement:  Copyright © 2018; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

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.


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