title: Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report.
abstract: Systemic capillary leak syndrome (SCLS), also called Clarkson's disease is rare and life-threatening disorder of unknown etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unexplained 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.
authors.affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.
authors.forename: Kun Moo
authors.initials: KM
authors.lastname: Choi
authors.affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.
authors.forename: Cheon Soo
authors.initials: CS
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.
authors.forename: Mi Hye
authors.initials: MH
authors.lastname: Kim
keywords: Hemoconcentration; Hypoalbuminemia; Systemic capillary leak syndrome