alexa Systemic capillary leak syndrome | Poland | 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 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 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 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: Poland anomaly appears with an incidence of about 1 in 30 000 live births and is three times more common in male than in female subjects. The syndrome was first described in 1841 by Alfred Poland, a British ophthalmologist, and typically comprises the combination of unilateral aplasia of the sternocostal head of the pectoralis major and an ipsilateral symbrachydactyly with a higher prevalence on the right side.

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