Capillary leak syndrome: Difference between revisions
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| DiseasesDB = 34090 | |||
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Revision as of 18:06, 25 January 2009
| Capillary leak syndrome | |
| DiseasesDB | 34090 |
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| MeSH | D019559 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Capillary Leak Syndrome or Systemic Capillary Leak Syndrome(SCLS). 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.
History
The syndrome was first described by Clarkson in 1960.
Symptoms
Edpisodes of
- Low blood pressure (hypotension)
- Hemoconcentration
- Hypoalbuminemia without albuminuria
- Generalized edema or anasarca
Natural hisotry
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.
Treatment
A few prophylactic treatments have been tried with variable results, principally terbutaline and theophylline.
Prognosis
Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
References
- Dorlands Medical Dictionary 30th Ed.
- Cancer.gov
- Case report in Oxford Journals
- Orphanet (2003)