A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case reportReportar como inadecuado




A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

Journal of Medical Case Reports

, 9:90

First Online: 28 April 2015Received: 14 October 2014Accepted: 13 February 2015DOI: 10.1186-s13256-015-0544-5

Cite this article as: Hirosaki, Y., Hayashidani, S., Ouchi, S. et al. J Med Case Reports 2015 9: 90. doi:10.1186-s13256-015-0544-5

Abstract

IntroductionIdiopathic systemic capillary leak syndrome is a rare and fatal disease due to the unexplained episodic attacks of capillary leakage of plasma from the intravascular into the interstitial space. The attack consists of three phases, a prodromal phase, peripheral leak phase and recruitment phase. During the peripheral leak phase, generalized edema, mainly in the trunk and extremities, with hemoconcentration and hypoalbuminemia occurs, while usually the visceral organs like lungs, brain, heart and kidneys seem not to be involved. Treatment of the acute phase is supportive, focusing on adequate but not overzealous fluid resuscitation, because pulmonary edema usually occurs in the recruitment phase.

Case presentationA 65-year-old Japanese woman was admitted to our hospital because of severe hypovolemic shock with metabolic acidosis and hemoconcentration and hypoalbuminemia. Although she was considered to be in the peripheral leak phase of idiopathic systemic capillary leak syndrome, which could not be diagnosed during the treatment, the generalized edema worsened further, severe flash pulmonary edema progressed rapidly after fluid resuscitation and she died. The autopsy showed generalized edema, especially alveolar pulmonary edema without endothelial apoptosis.

ConclusionsBecause hypovolemic shock and fatal pulmonary edema may progress rapidly together even in the peripheral leak phase of idiopathic systemic capillary leak syndrome, we should keep in mind this rare and fatal disease and recognize the pathophysiology to treat it effectively when the patient has hypovolemia with metabolic acidosis.

KeywordSystemic capillary leak syndrome Hypovolemic shock Pulmonary edema Autopsy findings AbbreviationsALPalkaline phosphatase

ALTalanine transaminase

ASTaspartate aminotransferase

cAMPcyclic adenosine monophosphate

CD8cluster of differentiation 8

CK-MBcreatine kinase MB

CPKcreatine phosphokinase

CTcomputed tomography

ECGelectrocardiogram

IgGimmunoglobulin G

IL-2interleukin 2

LDHlactate dehydrogenase

PEEPpositive end-expiratory pressure

SCLSidiopathic systemic capillary leak syndrome

TNF-alphatumor necrosis factor alpha

VEGFvascular endothelial growth factor

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Autor: Yuri Hirosaki - Shunji Hayashidani - Sayako Ouchi - Tukasa Ohshima - Ryuji Nakano - Hideo Yamamoto

Fuente: https://link.springer.com/







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