Capillary leak syndrome: Difference between revisions
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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. | ||
==Natural History== | ==Natural History== | ||
Revision as of 19:35, 28 January 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Synonyms and Keywords: Systemic capillary leak syndrome; SCLS; Clarkson's disease.
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.
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%.[1][2] 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.[3] 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.
Diagnosis
Symptoms
Generalized swelling may be present.
Physical Examination
Vitals
Skin
Laboratory Studies
- Hemoconcentration
- Hypoalbuminemia without albuminuria