Clinical analysis of 34 cases with sepsis and systemic capillary leak syndrome / 中华外科杂志

Objective@#To study the clinical characteristics of sepsis with systemic capillary leak syndrome(SCLS) and to evaluate the therapeutic effect and clinical significance of fluid therapy adjusted timely in these patients.@*Methods@#The clinical data of 34 patients with sepsis and SCLS in the Department of Hepatobiliary Surgery ICU of General Hospital of People′s Liberation Army General Hospital from July 2014 to January 2016 were retrospectively analyzed.There were 21 males and 13 females, aged from 21 to 74 years, with an average age of 56.3 years.Primary disease as follows: 18 cases with severe acute pancreatitis, 7 postoperative cases of subtotal hepatectomy, 5 postoperative cases of pancreatoduodenectomy, 4 postoperative cases of cholelithiasis.These patients were divided into survival group and death group according to their 28-day survival status.The clinical data including C-reactive protein(CRP), platelets (PLT), brain natriuretic peptide (BNP), the level of arterial blood lactic acid(LAC), oxygenation index(PaO2/FiO2, OI), net fluid balance(NFB) and norepinephrine dosage(NE) were collected and compared between two groups at three different intervals(day 1-3, day 4-6, day 7-9). The measurement data and numeration data were statistically analyzed with t test and χ2 test respectively to explore the inherent characteristics of the disease evolution and its clinical significance.@*Results@#The survival group (n=23)and the death group(n=11)had no significant difference in the characteristics of basic clinical characters.The condition of the survival group and the death group were both in progress in 1-3 days period manifested as increased CRP(t=-0.473, P=0.640) and BNP levels(t=0.140, P=0.895), decreased PLT counts(t=-0.505, P=0.620) in the inflammatory response, decreased LAC(t=-1.008, P=0.320) and OI level (t=-2.379, P=0.020)in tissue perfusion index, and positive fluid balance(NFB: t=0.910, P=0.370), required NE(t=-0.853, P=0.400) to maintain effective perfusion pressure with systemic edema in both groups.There was no significant difference of all these clinical parameters between the two groups.The patients′ condition of the survival group reached a plateau phase, whereas all relative indicators of the death group implied significant aggravation and deterioration of systemic infection(CRP: t=-3.438, P=0.000; PLT: t=1.649, P=0.110; BNP: t=-10.612, P=0.000), tissue perfusion (LAC: t=-11.305, P=0.000; OI: t=2.743, P=0.010)and tissue edema NFB(t=-4.257, P=0.000) and NE(t=-7.956, P=0.000) in 4-6 days period.In the last 7-9 days period the patients′ condition of the survival group took a turn for improvement, yet the condition of the death group continued to deteriorate, refractory septic shock developed and multiple organ dysfunction syndrome followed afterwards inevitably(CRP: t=-10.036, P=0.000; PLT: t=6.061, P=0.000; BNP: t=-10.119, P=0.000; LAC: t=-24.466, P=0.000; OI: t=13.443, P=0.010; NFB: t=-8.345, P=0.000; NE: t=-7.121, P=0.000).@*Conclusions@#The condition of patient with sepsis and SCLS would be improved markedly at the critical turning point around 7-9 days period since the effective systemic treatment began.If the infection does not be significantly constrolled and SCLS still remains in a sustained extravasation period in 7-9 days, the prognosis of these patients may be worse and the mortality may be higher than that of the patients mentioned before.

A Case of Systemic Capillary Leak Syndrome Presenting as Septic Shock / 대한내과학회지

Systemic capillary leak syndrome is a rare disease characterized by life-threatening attacks of reversible plasma extravasation and vascular collapse accompanied by hypotension, hemoconcentration, and hypoalbuminemia. A 36-year-old woman was admitted to this hospital with a fever, along with symptoms consistent with an upper respiratory tract infection and hypotension. Initial laboratory tests revealed several abnormal findings, including an elevated leukocyte count and hematocrit, hypoalbuminemia, and acute renal failure. Here, we report a case of successful treatment of systemic capillary leak syndrome, which can be difficult to distinguish from septic shock.

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

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.

A Case of Chronic Renal Failure Associated with Systemic Capillary Leak Syndrome / 영남의대학술지

Systemic capillary leak syndrome (SCLS) is an unusual entity characterized by hypovolemic shock, hemoconcentration, and hypo-albuminemia associated with paraproteinemia as a result of marked capillary hyperpermeability. Complications of this syndrome can include compartment syndromes, pulmonary edema, thrombosis, and acute kidney injury. This paper reports a case of severe SCLS accompanied by acute tubular necrosis caused by hypoperfusion and myoglobinuria secondary to rhabdomyolysis, which resulted in chronic kidney disease that necessitated hemodialysis. However, there have been rare data of residual end-organ damage after acute attacks in Korea. Therefore, this paper reports a case of complicated SCLS enough to hemodialysis and that developed into chronic kidney disease.

Recurrent Systemic Capillary Leak Syndrome Responding to the Corticosteroid Therapy / 대한신장학회지

Systemic capillary leak syndrome (SCLS) is a rare devastating condition that is caused by unexplained marked capillary hyperpermeability, resulting in hypovolemic shock, hemoconcentration, and hypoproteinemia. Most patients have prodromal symptoms such as non-specific gastrointestinal symptoms, myalgia, or dizziness. In the current case, we observed the patient with recurrent SCLS show perivascular lymphocytic infiltration on skin biopsy and eosinophilic infiltrates on random biopsies from duodenum to colon. This finding might be due to distinct eosinophilic enterocolitis or secondary to SCLS. However, there has been rare data for the prevention of the recurrent attack of SCLS, although the mortality is high. Therefore, we report a case of frequently relapsing SCLS responding to the corticosteroid therapy.

Two Cases of Systemic Capillary Leak Syndrome that were Treated with Pentastarch

Systemic capillary leak syndrome (SCLS) is a condition that's caused by the shift of fluid and protein from the intravascular space to the interstitial space as a result of repetitive episodes of capillary hyperpermeability. The pathogenesis of SCLS is still unclear, but there's recently been a report showing this syndrome in association with monoclonal gammopathy. This syndrome can be a fatal disease because cardiovascular collapse can occur in the initial capillary leak phase. Although theophylline, diuretics, terbutaline, steroids, calcium antagonist, Ginkgo biloba extracts and plasmapheresis have been suggested as medication, none of them have been proven to be effective. Considering that this disease is self-limiting, conservative treatment in the acute phase is believed to be very important. Because hypoalbuminemia is very a common manifestation of SCLS, Pentastarch, which has a higher molecular weight than albumin, could be efficient to prevent cardiovascular collapse. We used 10% Pentastarch during the acute SCLS attacks of 2 patients and the patients both showed a dramatic response. Pentastarch may be helpful to treat SCLS in its initial capillary leak phase by the elevating blood pressure, and this might contribute to somewhat decreasing the acute mortality of SCLS.

A Case of Systemic Capillary Leak Syndrome Following Upper Respiratory Tract Infection / 대한신장학회잡지

Systemic capillary leak syndrome is a rare condition characterized by recurrent episodes of generalized edema and severe hypotension associated with paraproteinaemia. We experienced a case of recurrent systemic capillary leak syndrome. A 30-year-old woman had flu-like syndrome with fever, chill, and sore throat. Two days later, she presented progressive generalized edema and weight gain of 4 kg. Biological examination showed hemoconcentration, leukocytosis, and hypoalbuminemia without proteinuria. A radiograph of the chest showed pleural effusion. She had no evidence of heart failure, renal failure, or a protein losing enteropathy, and no abnormalities of liver function. Anti-neuclear antibody and antibodies to ds-DNA were negative. C3 and C4 were decreased. Serum protein electrophoresis showed oligoclonal banding consisting of IgG bands. Bone marrow biopsy was normal and there was no Bence-Jones protein in urine. She experienced a progressive reduction in her weight and a resolution of the peripheral edema without specific treatment. Five months later, she was readmitted with severe episode of shock. After a total of 10 L of fluid was administered intravenously, pulmonary edema developed. Intravenous steroid and theophylline were given which resulted in clinical improvement, accompanied by diuresis. Oral theophylline has been administered for prevention of this crisis up to the present time.

A Case of Systemic Capillary Leak Syndrome in a Child

Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.