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==Historical Perspective==
==Historical Perspective==
The [[syndrome]] was first described by B. Clarkson in 1960, after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.
The [[syndrome]] was first described by B. Clarkson in 1960, after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.
==[[Capillary leak syndrome pathophysiology|Pathophysiology]]==
The pathogenesis of capillary leak syndrome remain unclear, however, '''[https://www.wikidoc.org/index.php/Cytokines cytokines]''', '''[https://www.wikidoc.org/index.php/Vascular_endothelial_growth_factor vascular endothelial growth factor]''' (VEGF), '''[https://www.wikidoc.org/index.php/Leukotrienes leukotrienes]''', and '''complement''' play an important role.  During each episode of hyperpermeability of the vasculature, there is an increase of interleukin 2 receptor-positive peripheral blood M-cell count, as well as apoptosis of the endothelial cells.


<br />
==[[Capillary leak syndrome differential diagnosis|Differentiating Capillary leak syndrome from other Diseases]]==
As capillary leak syndrome has overlapping clinical and laboratory findings with various other conditions, it should be considered as the diagnosis of exclusion.  Several conditions that may be considered as the differential diagnoses of capillary leak syndrome include [https://www.wikidoc.org/index.php/Polycythemia%20vera polycythemia vera], [https://www.wikidoc.org/index.php/Hereditary%20angioedema hereditary angioedema], Gleich syndrome, [https://www.wikidoc.org/index.php/Adrenal%20insufficiency adrenal insufficiency], [https://www.wikidoc.org/index.php/Mast%20cell%20tumor mast cell disease], [https://www.wikidoc.org/index.php/Acute%20gastroenteritis acute gastroenteritis], [https://www.wikidoc.org/index.php/Carcinoid%20syndrome carcinoid syndrome], [https://www.wikidoc.org/index.php/Primary%20amyloidosis primary amyloidosis], [https://www.wikidoc.org/index.php/Chronic%20heart%20failure chronic heart failure], [https://www.wikidoc.org/index.php/Sepsis sepsis], and [https://www.wikidoc.org/index.php/Toxic%20shock%20syndrome toxic shock syndrome].
 
==[[Capillary leak syndrome epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Capillary leak syndrome risk factors|Risk Factors]]==
 
==[[Capillary leak syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
==Diagnosis==
 
[[Capillary leak syndrome history and symptoms|History and Symptoms]] | [[Capillary leak syndrome physical examination|Physical Examination]] | [[Capillary leak syndrome laboratory findings|Laboratory Findings]] | [[Capillary leak syndrome other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==
[[Capillary leak syndrome medical therapy|Medical Therapy]] | [[Capillary leak syndrome prevention|Prevention]] | [[Capillary leak syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Capillary leak syndrome future or investigational therapies|Future or Investigational Therapies]]
===Prevention===
===Prevention===
A few [[prophylactic]] treatments have been tried with variable results, principally [[terbutaline]] and [[theophylline]].
A few [[prophylactic]] treatments have been tried with variable results, principally [[terbutaline]] and [[theophylline]].

Revision as of 07:25, 7 August 2022

Capillary leak syndrome Microchapters

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Historical Perspective

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Epidemiology and Demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Overview

Capillary leak syndrome is a rare medical condition characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for a leakage of fluid from the circulatory system to the interstitial space, resulting in a dangerous hypotension, hemoconcentration, and hypoalbuminemia. It is a life-threatening illness because each episode has the potential to cause damage to, or the failure of, vital organs due to limited perfusion. It is often misdiagnosed as polycythemia, polycythemia vera or sepsis.

Historical Perspective

The syndrome was first described by B. Clarkson in 1960, after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.

Pathophysiology

The pathogenesis of capillary leak syndrome remain unclear, however, cytokines, vascular endothelial growth factor (VEGF), leukotrienes, and complement play an important role. During each episode of hyperpermeability of the vasculature, there is an increase of interleukin 2 receptor-positive peripheral blood M-cell count, as well as apoptosis of the endothelial cells.

Differentiating Capillary leak syndrome from other Diseases

As capillary leak syndrome has overlapping clinical and laboratory findings with various other conditions, it should be considered as the diagnosis of exclusion. Several conditions that may be considered as the differential diagnoses of capillary leak syndrome include polycythemia vera, hereditary angioedema, Gleich syndrome, adrenal insufficiency, mast cell disease, acute gastroenteritis, carcinoid syndrome, primary amyloidosis, chronic heart failure, sepsis, and toxic shock syndrome.

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Prevention

A few prophylactic treatments have been tried with variable results, principally terbutaline and theophylline.

References


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