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Systemic Capillary Leak Syndrome (systemic + capillary_leak_syndrome)

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Medical Sciences100%

Selected Abstracts

A case report of systemic capillary leak syndrome (Clarkson's disease)

ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 5 2010
J. HOLLENBERG
Systemic capillary leak syndrome (SCLS) is a very rare disorder also known as Clarkson's disease. The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration, hypoalbuminemia, hypovolemia and shock. We describe a 41-year-old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia. Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock. He received a combination of prophylactic treatment with theophylline, ,-agonists, immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest. Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload. SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found. The pathogenesis is unknown and the attacks may be lethal. [source]

Systemic capillary leak syndrome resulting in compartment syndrome and the requirement for a surgical airway

ANAESTHESIA, Issue 6 2009
J. Perry
Summary We report on a case of systemic capillary leak syndrome associated with a monoclonal band on plasma electrophoresis. In our patient hospital admission was precipitated by ischaemic pain in the left lower limb, associated with polycythaemia, renal failure and hypovolaemic shock. Fluid resuscitation, venesection and renal replacement therapy were instituted but a compartment syndrome developed necessitating surgery. Failure of tracheal intubation resulted in the requirement for a surgical airway. Despite surgical and resuscitative efforts the outcome was fatal from hypovolaemia and hyperkalaemia. We aim to highlight the difficulties in managing this condition and to remind healthcare workers to include it in the differential diagnoses for patients presenting with polycythaemia; in particular polycythaemic patients with a monoclonal band on plasma electrophoresis. [source]

A case report of systemic capillary leak syndrome (Clarkson's disease)

ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 5 2010
J. HOLLENBERG
Systemic capillary leak syndrome (SCLS) is a very rare disorder also known as Clarkson's disease. The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration, hypoalbuminemia, hypovolemia and shock. We describe a 41-year-old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia. Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock. He received a combination of prophylactic treatment with theophylline, ,-agonists, immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest. Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload. SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found. The pathogenesis is unknown and the attacks may be lethal. [source]

Systemic capillary leak syndrome resulting in compartment syndrome and the requirement for a surgical airway

ANAESTHESIA, Issue 6 2009
J. Perry
Summary We report on a case of systemic capillary leak syndrome associated with a monoclonal band on plasma electrophoresis. In our patient hospital admission was precipitated by ischaemic pain in the left lower limb, associated with polycythaemia, renal failure and hypovolaemic shock. Fluid resuscitation, venesection and renal replacement therapy were instituted but a compartment syndrome developed necessitating surgery. Failure of tracheal intubation resulted in the requirement for a surgical airway. Despite surgical and resuscitative efforts the outcome was fatal from hypovolaemia and hyperkalaemia. We aim to highlight the difficulties in managing this condition and to remind healthcare workers to include it in the differential diagnoses for patients presenting with polycythaemia; in particular polycythaemic patients with a monoclonal band on plasma electrophoresis. [source]