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

Choi KM, Park CS, Kim MH - Korean J Hepatobiliary Pancreat Surg (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

Serum protein electrophoresis.
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Figure 3: Serum protein electrophoresis.

Mentions: The patient was taken to the surgical intensive care unit and a chest radiograph just after the surgery revealed diffuse bilateral infiltrates, without cardiomegaly (Fig. 1B). Mechanical ventilation with pressure support mode was applied. Immediate postoperative laboratory studies showed hemoglobin 18.1 g/dl, hematocrit 51.9%, leukocyte count 23,600/ul, total protein 2.8 g/dl, and albumin 1.1 g/dl. Critical medicine doctor presumptively diagnosed systemic capillary leak syndrome (SCLS) and 20% albumin, hydrocortisone 300 mg/day and theophylline 400 mg/day, human immune globulin G (IVIG) 1 g/kg/day were immediately administered, intravenously. On the postoperative day 3, total protein/albumin and Hb/Hct levels turned to 5.6/3.3 g/dl, 11.8 g/dl/35.1%, respectively, but creatine phosphokinase (CPK) was elevated to 1,897 U/L (normal range: 50-250 U/L), procalcinotnin 14 ng/ml, and CRP 21.53 mg/dl. Immunoglobulin quantification study showed that the complements, C3 and 4 were normal, the immunoglobulin (Ig) G 406.5 mg/dl (normal range: 694-1,618) and IgM 40 mg/dl (normal range: 60-263) were decreased, and however, Ig E was 681.13 IU/ml (normal range: <120), which was elevated. Ig A was within the normal range. Serum electrophoresis showed that total protein was within normal limit (6.3 g/dl) and alpha-1-globulin fraction was increased, and there was no abnormal band (Fig. 3). About the time when pulmonary edema has improved considerably, the patient showed abnormal eye ball movements as well as the generalized seizure on the postoperative day 7. A portable electroencephalography (EEG) was taken, which showed suggestive of severe diffuse cerebral dysfunction. Subsequently performed enhanced computed tomography scan and magnetic resonance imaging of the head showed diffuse brain atrophy and ischemic change in the periventricular deep white matter. In the meantime, the final report of surgical pathology was an adenocarcinoma arisen from intraductal papillary neoplasm of the distal common bile duct with pancreatic, perineural invasions, but clear resection margins. Two out of 28 nodes were metastatic regional lymph nodes. On the postoperative day 14, her pulmonary condition has fully improved; however, unfortunately, she is still in bed more than one year because of brain injury.


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

Choi KM, Park CS, Kim MH - Korean J Hepatobiliary Pancreat Surg (2014)

Serum protein electrophoresis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4492332&req=5

Figure 3: Serum protein electrophoresis.
Mentions: The patient was taken to the surgical intensive care unit and a chest radiograph just after the surgery revealed diffuse bilateral infiltrates, without cardiomegaly (Fig. 1B). Mechanical ventilation with pressure support mode was applied. Immediate postoperative laboratory studies showed hemoglobin 18.1 g/dl, hematocrit 51.9%, leukocyte count 23,600/ul, total protein 2.8 g/dl, and albumin 1.1 g/dl. Critical medicine doctor presumptively diagnosed systemic capillary leak syndrome (SCLS) and 20% albumin, hydrocortisone 300 mg/day and theophylline 400 mg/day, human immune globulin G (IVIG) 1 g/kg/day were immediately administered, intravenously. On the postoperative day 3, total protein/albumin and Hb/Hct levels turned to 5.6/3.3 g/dl, 11.8 g/dl/35.1%, respectively, but creatine phosphokinase (CPK) was elevated to 1,897 U/L (normal range: 50-250 U/L), procalcinotnin 14 ng/ml, and CRP 21.53 mg/dl. Immunoglobulin quantification study showed that the complements, C3 and 4 were normal, the immunoglobulin (Ig) G 406.5 mg/dl (normal range: 694-1,618) and IgM 40 mg/dl (normal range: 60-263) were decreased, and however, Ig E was 681.13 IU/ml (normal range: <120), which was elevated. Ig A was within the normal range. Serum electrophoresis showed that total protein was within normal limit (6.3 g/dl) and alpha-1-globulin fraction was increased, and there was no abnormal band (Fig. 3). About the time when pulmonary edema has improved considerably, the patient showed abnormal eye ball movements as well as the generalized seizure on the postoperative day 7. A portable electroencephalography (EEG) was taken, which showed suggestive of severe diffuse cerebral dysfunction. Subsequently performed enhanced computed tomography scan and magnetic resonance imaging of the head showed diffuse brain atrophy and ischemic change in the periventricular deep white matter. In the meantime, the final report of surgical pathology was an adenocarcinoma arisen from intraductal papillary neoplasm of the distal common bile duct with pancreatic, perineural invasions, but clear resection margins. Two out of 28 nodes were metastatic regional lymph nodes. On the postoperative day 14, her pulmonary condition has fully improved; however, unfortunately, she is still in bed more than one year because of brain injury.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

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.

No MeSH data available.


Related in: MedlinePlus