alexa Systemic capillary leak syndrome | Israel | PDF | PPT| Case Reports | Symptoms | Treatment

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Systemic Capillary Leak Syndrome

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  • Systemic capillary leak syndrome

    Disease pathophysiology: Systemic capillary leak syndrome is a rare disorder characterized by self-reversing episodes during which the endothelial Disease pathophysiology: Systemic capillary leak syndrome is a rare disorder characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and other blood components from the blood vessels into neighbouring body cavities and muscles. Systemic capillary leak syndrome usually consists of two phases i.e capillary leak phase and recruitment phase. This leads to swelling. Systemic capillary leak syndrome leads tohypotension, hemoconcentration, and hypoalbuminemia. It is a life threatning syndrome often misdiagnosed with polycythemia, polycythemia vera, or sepsis.cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and other blood components from the blood vessels into neighbouring body cavities and muscles. Systemic capillary leak syndrome usually consists of two phases i.e capillary leak phase and recruitment phase. This leads to swelling. Systemic capillary leak syndrome leads tohypotension, hemoconcentration, and hypoalbuminemia. It is a life threatning syndrome often misdiagnosed with polycythemia, polycythemia vera, or sepsis. 

  • Systemic capillary leak syndrome

     Treatment: Mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes. 

  • Systemic capillary leak syndrome

     The patient was diagnosed with SCLCS and treated with therbutaline and aminophylline. Research: Major research is been performed by NORD (National Organization of Rare Disorders) Statistics:The most common treatment modality during episodes is judicious use of intravenous fluids and vasopressors to maintain perfusion to the brain and other vital organs. Although no more than 100 cases of SCLS were reported in the literature from 1960 to 2006, the nonspecific nature of the presenting signs and symptoms and high mortality rate during episodes may have resulted in considerable underdiagnosis. Fifty new cases of SCLS were reported from 2006 to 2011, suggesting that there may be increased awareness of this disorder. The 5-year survival rate is ∼ 75%, and deaths are most commonly related to acute SCLS event.

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