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| '''For patient information, click [[Capillary leak syndrome (patient information)|here]]''' | | '''For patient information, click [[Capillary leak syndrome (patient information)|here]]''' |
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| {{CMG}} | | {{CMG}}; '''Associate Editor(s)-In-Chief:''' [https://www.wikidoc.org/index.php/User:Hassan_M M. Hassan, M.B.B.S] |
| {{Capillary leak syndrome}} | | {{Capillary leak syndrome}} |
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| '''''Synonyms and Keywords:''''' Systemic capillary leak syndrome; SCLS; Clarkson's disease. | | '''''Synonyms and Keywords:''''' Systemic capillary leak syndrome; SCLS; Clarkson's disease. |
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| ==Overview== | | ==[[Capillary leak syndrome overview|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 (biology)|interstitial space]], resulting in a dangerous [[hypotension]] (low blood pressure), [[hemoconcentration#Elevated|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]]. | |
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| ==Historical Perspective== | | ==[[Capillary leak syndrome historical perspective|Historical Perspective]]== |
| The [[syndrome]] was first described by B. Clarkson in 1960,<ref name="clarkson">{{cite journal |doi=10.1016/0002-9343(60)90018-8 |title=Cyclical edema and shock due to increased capillary permeability |year=1960 |last1=Clarkson |first1=Bayard |last2=Thompson |first2=David |last3=Horwith |first3=Melvin |last4=Luckey |first4=E.Hugh |journal=The American Journal of Medicine |volume=29 |issue=2 |pmid=13693909 |pages=193–216}}</ref> 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.<ref name="druey">{{cite journal |first1=Kirk M. |last1=Druey |first2=Philip R. |last2=Greipp |title=Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome |journal=Annals of Internal Medicine |pmid=20643990 |pages=90–8 |doi=10.1059/0003-4819-153-2-201007200-00005 |pmc=3017349 |year=2010 |volume=153 |issue=2}}</ref><ref name="kapoor">{{cite journal |pages=905–12 |doi=10.4065/mcp.2010.0159 |pmc=2947962 |title=Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience |year=2010 |last1=Kapoor |first1=Prashant |last2=Greipp |first2=Patricia T. |last3=Schaefer |first3=Eric W. |last4=Mandrekar |first4=Sumithra J. |last5=Kamal |first5=Arif H. |last6=Gonzalez-Paz |first6=Natalia C. |last7=Kumar |first7=Shaji |last8=Greipp |first8=Philip R. |journal=Mayo Clinic Proceedings |volume=85 |issue=10 |pmid=20634497}}</ref>
| | ==[[Capillary leak syndrome pathophysiology|Pathophysiology]]== |
| | ==[[Capillary leak syndrome causes|Causes]]== |
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| ==Natural History== | | ==[[Capillary leak syndrome differential diagnosis|Differentiating Capillary leak syndrome from other Diseases]]== |
| The episode usually consists of two phases
| | ==[[Capillary leak syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
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| 1. The capillary leak phase (1-4 days)
| | ==[[Capillary leak syndrome risk factors|Risk Factors]]== |
| The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are [[abdominal pain]], [[nausea]], generalized edema and [[hypotension]] that may result in cardiopulmonary collapse. [[Acute renal failure]] is due to [[acute tubular necrosis]] consequent to [[hypovolemia]] and [[rhabdomyolysis]].
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| 2. Recruitment of the interstitial fluid
| | ==[[Capillary leak syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with [[polyuria]] and [[pulmonary edema]] often occur. Edema may be more severe due to massive fluid supply in the initial phase. It's necessary to monitor the patient in order to switch to depletion treatment with [[diuretics]] or [[hemofiltration]].
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| ==Prognosis== | |
| Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
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| In the Mayo Clinic’s experience, the median survival of 25 patients that were followed over 30 years (counting only SCLS-related deaths) was approximately 15 years, and their 5-year survival rate was 76%.<ref name="druey"/><ref name="kapoor"/> In European experience, the 5-year post-diagnosis survival rate was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not.<ref name="gousseff" /> However, better identification and management of this condition appears to be resulting in lower mortality and improving survival and quality-of-life results as of late.
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| ==Diagnosis== | | ==Diagnosis== |
| ===Symptoms===
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| [[Generalized swelling]] may be present.
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| ===Physical Examination===
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| ====Vitals====
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| * [[Low blood pressure]] ([[hypotension]])
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| ====Skin====
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| Generalized [[edema]] or [[anasarca]]
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| ===Laboratory Studies===
| | [[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]] |
| * Hemoconcentration
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| * [[Hypoalbuminemia]] without [[albuminuria]]
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| ==Case Reports== | | ==Treatment== |
| *[http://ndt.oxfordjournals.org/cgi/content/full/17/3/492 Case report in Oxford Journals]
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| ==Sources==
| | [[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]] |
| * Dorlands Medical Dictionary 30th Ed.
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| * [http://www.cancer.gov/Templates/db_alpha.aspx?CdrID=426421 Cancer.gov]
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| * [http://www.orpha.net/static/GB/capillary_leak_syndrome.html Orphanet (2003)]
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| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[Capillary leak syndrome case study one|Case #1]] |
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| [[Category:Syndromes]] | | [[Category:Syndromes]] |
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