alexa Systemic capillary leak syndrome | Spain | 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 cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and otherblood 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 to hypotension, 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 likemethylprednisolone, 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. 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: Mean age of onset is 45 and pediatric or geriatric cases are rare.

  • Systemic capillary leak syndrome

    Statastics: Among the total population of the Spain, there are very few members who are suffring from Systemic capillary leak syndrome as it is a very rare disease. They last days or weeks, can be of variable severity, from grade 1 (hypotension responding to oral hydration) to grade 4 (fatal attacks), and have a 3-step evolution.

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