Capillary leak syndrome: Difference between revisions
No edit summary |
|||
| Line 23: | Line 23: | ||
==Historical Perspective== | ==Historical Perspective== | ||
The [[syndrome]] was first described by Clarkson in 1960. | The [[syndrome]] was first described by Clarkson in 1960. | ||
==Natural History== | |||
The episode usually consists of two phases | |||
1. The capillary leak phase (1-4 days) | |||
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]]. | |||
2. Recruitment of the interstitial fluid | |||
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]]. | |||
==Prognosis== | |||
Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality. | |||
==Diagnosis== | ==Diagnosis== | ||
| Line 37: | Line 49: | ||
* Hemoconcentration | * Hemoconcentration | ||
* [[Hypoalbuminemia]] without [[albuminuria]] | * [[Hypoalbuminemia]] without [[albuminuria]] | ||
==Treatment== | ==Treatment== | ||
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]]. | ||
== | ==Case Reports== | ||
*[http://ndt.oxfordjournals.org/cgi/content/full/17/3/492 Case report in Oxford Journals] | |||
== | ==Sources== | ||
* Dorlands Medical Dictionary 30th Ed. | * Dorlands Medical Dictionary 30th Ed. | ||
* [http://www.cancer.gov/Templates/db_alpha.aspx?CdrID=426421 Cancer.gov] | * [http://www.cancer.gov/Templates/db_alpha.aspx?CdrID=426421 Cancer.gov] | ||
* [http://www.orpha.net/static/GB/capillary_leak_syndrome.html Orphanet (2003)] | * [http://www.orpha.net/static/GB/capillary_leak_syndrome.html Orphanet (2003)] | ||
==References== | |||
{{ | {{Reflist|2}} | ||
[[Category:Syndromes]] | [[Category:Syndromes]] | ||
Revision as of 15:14, 11 September 2011
| Capillary leak syndrome | |
| DiseasesDB | 34090 |
|---|---|
| MeSH | D019559 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and Keywords: Systemic Capillary Leak Syndrome, SCLS
Overview
Capillary Leak Syndrome is a rare medical condition where the number and size of the pores in the capillaries are increased which leads to a leakage of fluid from the blood to the interstitial fluid, resulting in dangerously low blood pressure (hypotension), edema and multiple organ failure due to limited perfusion.
Historical Perspective
The syndrome was first described by Clarkson in 1960.
Natural History
The episode usually consists of two phases
1. The capillary leak phase (1-4 days) 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.
2. Recruitment of the interstitial fluid 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.
Prognosis
Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
Diagnosis
Symptoms
Generalized swelling may be present.
Physical Examination
Vitals
- Low blood pressure (hypotension)
Skin
Laboratory Studies
- Hemoconcentration
- Hypoalbuminemia without albuminuria
Treatment
A few prophylactic treatments have been tried with variable results, principally terbutaline and theophylline.
Case Reports
Sources
- Dorlands Medical Dictionary 30th Ed.
- Cancer.gov
- Orphanet (2003)