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KMID : 0870420140180010038
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Korean Journal of Hepato-Biliary-Pancreatic Surgery
2014 Volume.18 No. 1 p.38 ~ p.41
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Systemic capillary leak syndrome (Clarkson’sdisease) during elective pylorus-preserving pancreaticoduodenectomy: case report
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Choi Kun-Moo
Park Cheon-Soo
Kim Mi-Hye
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Abstract
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Systemic capillary leak syndrome (SCLS), also called Clarkson’sdisease is rare and life-threatening disorder of un-known etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unex-plained capillary leakage from the intravascular to the interstitial space, which has been estimated up to 70% of the intravascular volume, is the proposed mechanism. Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established. The mortality rate ranges from 30% to 76%. In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012. We describe a case of severe SCLS that suddenly occurred and rapidly progressed during pylorus preserving pancreaticoduodenectomy and review the literature.
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KEYWORD
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Systemic capillary leak syndrome, Hemoconcentration, Hypoalbuminemia
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