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Case Reports
. 2014 Feb;18(1):38-41.
doi: 10.14701/kjhbps.2014.18.1.38. Epub 2014 Feb 24.

Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report

Kun Moo Choi  1 , Cheon Soo Park  1 , Mi Hye Kim  2
Affiliations
Case Reports

Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report

Kun Moo Choi et al. Korean J Hepatobiliary Pancreat Surg. 2014 Feb.

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.

Keywords: Hemoconcentration; Hypoalbuminemia; Systemic capillary leak syndrome.

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Figures

Fig. 1
Fig. 1
(A) Preoperative chest x-ray. (B) Postoperative chest x-ray.
Fig. 2
Fig. 2
Episode of intraoperative hypotension (arrow).
Fig. 3
Fig. 3
Serum protein electrophoresis.
See this image and copyright information in PMC

References

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