Acute renal failure associated with systemic capillary leak syndrome

2000 ◽  
Vol 4 (3) ◽  
pp. 261-265
Author(s):  
K. Fukunari ◽  
M. Nishimura ◽  
F. Taguchi ◽  
A. Yamada ◽  
S. Okamatsu
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Kwadwo Kyeremanteng ◽  
Gianni D’Egidio ◽  
Cynthia Wan ◽  
Alan Baxter ◽  
Hans Rosenberg
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Compartment Syndrome ◽  
Single Case ◽  
Rare Complication ◽  
Clinical Syndrome ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Fluid Extravasation

Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome.Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain.Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion) and respiratory (ventilator) support in the ICU was provided until resolution of intravascular fluid extravasation.Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients.

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

2012 ◽  
Vol 29 (2) ◽  
pp. 145 ◽  
Author(s):  
Seon Ha Baek ◽  
Nara Shin ◽  
Hyo Jin Kim ◽  
Mi Yeun Han ◽  
Dong-Ju Choi ◽  
...  
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Blood ◽  
2012 ◽  
Vol 119 (18) ◽  
pp. 4321-4332 ◽  
Author(s):  
Zhihui Xie ◽  
Chandra C. Ghosh ◽  
Roshni Patel ◽  
Shoko Iwaki ◽  
Donna Gaskins ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Case Series ◽  
Fiber Formation ◽  
Capillary Leak Syndrome ◽  
Actin Stress Fiber ◽  
Vascular Endothelial ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Humoral Factors ◽  
Clarkson Disease

Abstract The systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient episodes of hypotensive shock and anasarca thought to arise from reversible microvascular barrier dysfunction. Although the high prevalence of a monoclonal gammopathy of unknown significance in SCLS suggests a pathogenic contribution of endogenous immunoglobulins, the mechanisms of vascular hyperpermeability remain obscure. Herein, we report clinical and molecular findings on 23 patients, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from patients during remission, to human microvascular endothelial cells caused vascular endothelial cadherin internalization, disruption of interendothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin, one promising therapy for SCLS, mitigated the permeability effects of episodic sera. Consistent with the presence of endogenous, nonimmunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Ab-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF Ab (bevacizumab) yielded less interpretable results, probably because of endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which nonimmunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.

scholarly journals A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Aysun Ata ◽  
Samim Özen ◽  
Damla Gökşen ◽  
Neslihan Edeer Karaca ◽  
Güzide Aksu ◽  
...  
Keyword(s):  
Urine Analysis ◽  
Early Recognition ◽  
Recovery Period ◽  
Capillary Leak Syndrome ◽  
Ivig Treatment ◽  
Acid Decarboxylase ◽  
Diabetic Patients ◽  
Healthy Children ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeability followed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy was admitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, and oral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchy edema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L, hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourth day, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immunoglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased on the fifth day, and the patient was deemed clinically well. There was no compartment syndrome, rhabdomyolysis, or pulmonary edema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) and enterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serum glucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive. However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit, 22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis, there was no ISCLS attack. Conclusion. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare in childhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of the disease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to the literature since there have been no pediatric diabetic patients with ISCLS reported.

Masquerading as Septic Shock: Idiopathic Systemic Capillary Leak Syndrome

CHEST Journal ◽  
2014 ◽  
Vol 146 (4) ◽  
pp. 295A
Author(s):  
Kymberly McDonald ◽  
Vanessa Yap ◽  
Stephen Meng ◽  
Prashant Grover ◽  
Daniel Gerardi
Keyword(s):  
Septic Shock ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Systemic Capillary Leak Syndrome (Clarkson Syndrome) in Cancer Patients: A Systematic Review

2018 ◽  
Vol 7 (11) ◽  
pp. 418 ◽  
Author(s):  
Jae Shin ◽  
Keum Lee ◽  
I. Lee ◽  
Ji Oh ◽  
Dong Kim ◽  
...  
Keyword(s):  
Cancer Patients ◽  
Case Reports ◽  
Laboratory Data ◽  
Capillary Leak Syndrome ◽  
Treatment Modalities ◽  
Capillary Leak ◽  
Contributing Factors ◽  
Systemic Capillary Leak Syndrome ◽  
Increased Risk ◽  
Anti Cancer

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by shock caused by capillary hyperpermeability. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. The aim of the study was to analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to identify contributing factors leading to mortality of SCLS in cancer. We searched MEDLINE (inception to July 2018) and of 4612 articles, we identified 62 case reports on SCLS associated with cancer or cancer-related drugs in a total of 53 articles. SCLS was associated with cancer itself in 43.6%, with anti-cancer agents in 51.6% and bone marrow transplantation (BMT) in 4.8%. Among anti-cancer agents, granulocyte-colony stimulating factor (G-CSF) was the most frequently associated drug (14.6%), followed by interleukin (IL)-2 (11.4%). The most common associated malignancies were hematologic (61.3%) with non-Hodgkin lymphoma (22.7%) and multiple myeloma (12.9%) being the leading causes. Common symptoms and signs included dyspnea (27.4%), edema (67.7%), hypotension (32.2%), pleural effusion (29.0%), ascites (22.7%), oliguria (22.7%), and weight gain (21.0%). Patients with SCLS were treated with steroids (59.7%), volume replacement (33.8%), diuretics (24.2%), inotropes (9.6%), methylxanthines (12.8%), β2 agonists (4.8%), while intravenous immunoglobulins (IVIG) were administered in 2 patients (3.2%) only. Among sixteen deaths during follow-up, four were directly attributed to SCLS. Hematologic malignancies were associated with an increased risk for mortality (hazard ratio (HR) 8.820, 95% confidence interval (CI) 1.126–69.063, p = 0.038). Taken together, SCLS can be one important adverse event in cancer patients and careful monitoring of fluid volume is required in the management of SCLS.

Sign in / Sign up

Export Citation Format

Share Document