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Systemic Capillary Leak Syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management

DOI: 10.1186/1757-7241-18-38

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Abstract:

Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by unexplained, often recurrent, non sepsis-related episodes of increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy (IgG class monoclonal gammopathy predominates, with either kappa or lambda light chains) are the characteristic laboratory findings. SCLS was first described in 1960 by Clarkson et al. [1]. Common clinical manifestations of SCLS are diffuse swelling, weight gain, renal shut-down and hypovolemic shock. Here we present a patient who suffered from SCLS with hypovolemic shock and compartment syndrome of both lower legs and thighs. In this patient volume and catecholamine management was guided using transpulmonary thermodilution.A 41-year-old male with compartment syndrome of both lower legs and thighs was transferred to our intensive care unit (ICU) (hospital B) after emergency decompressive fasciotomy in another hospital (hospital A) the previous day (fig. 1).On admission to hospital A the previous day the patient had presented with severe muscle pain in the legs and a 2-week history of flu-like illness and sore throat with fever up to 39°C, which had been treated with moxifloxacin for several days. On initial physical examination signs of massive dehydration were present (heart rate 102/min; blood pressure 65/50 mmHg, temperature 37.1°C).Extensive fluid resuscitation was initiated (15 L on hospital day 1). Previous medical history was unremarkable. The patient was working as a policeman and had been to Italy three weeks prior to admission. He reported playing in a football tournament one week previously.Blood biochemistry indicated severe hemoconcentration (hemoglobin 22.3 g/dL, hematocrit 60.4%), hypoproteinemia (serum total protein 2.3 g/dL) and acute kidney failure (creatine 1.6 mg/dL, blood urea nit

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