scholarly journals Autonomic biomarkers of shock in idiopathic systemic capillary leak syndrome

PLoS ONE ◽  
2021 ◽  
Vol 16 (6) ◽  
pp. e0251775
Author(s):  
Maddalena Alessandra Wu ◽  
Emanuele Catena ◽  
Antonio Castelli ◽  
Roberto Rech ◽  
Beatrice Borghi ◽  
...  
Keyword(s):  
Heart Rate ◽  
Acute Phase ◽  
Systolic Arterial Pressure ◽  
Recovery Phase ◽  
Capillary Leak Syndrome ◽  
Haemodynamic Instability ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Baroreflex Control ◽  
Distributive Shock

Objective The term Idiopathic Systemic Capillary Leak Syndrome (ISCLS) refers to an uncommon condition of severe distributive shock, resulting from an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. We hypothesise that the autonomic nervous system (ANS) fails in regulating the response to hypovolemia in acute ISCLS and that ANS variables characterise the progression to the recovery. Design Prospective cohort study of patients admitted to ICU for severe ISCLS flares. Setting Single, referral center in Italy for ISCLS. Patients Analysis of cardiovascular signals recorded during seven severe ISCLS attacks and one prodromal period in five patients. Interventions ANS was studied non-invasively by means of heart rate variability (HRV) and blood pressure variability analysis, as an estimation of vagal and sympathetic modulation directed to the heart and vessels. Heart rate and systolic arterial pressure (SAP) variability were also used to assess baroreflex sensitivity. ANS variables were measured during the subsequent phases which characterise ISCLS flares, namely the acute phase, the post-acute phase, and the recovery phase. Measurements and main results HRV was severely depressed during the acute phase accounting for the loss of ANS modulation during massive capillary extravasation. This phase was characterised by shock and impaired baroreflex control, which allowed SAP to oscillate driven by respiratory activity. Impending shock and transition from shock to a post-acute phase were marked by change of baroreflex spectral variables. The baroreflex control was fully restored during recovery. Conclusions ANS modulation and baroreflex control are severely impaired during the acute haemodynamic instability which characterises ISCLS crises and their progressive restoration may be a clue of improvement. ANS indices during ISCLS flares might serve as useful biomarkers, able to timely announce the transition from one phase to the subsequent one, thus helping to adapt therapy accordingly.

scholarly journals Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase

2017 ◽  
Vol 07 (02) ◽  
pp. 094-096 ◽  
Author(s):  
Ashley Bjorklund ◽  
Gwenyth Fisher ◽  
Anna Sofi Asmundsson
Keyword(s):  
Intravenous Immunoglobulin ◽  
Acute Phase ◽  
Clinical Course ◽  
Pediatric Intensive Care ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Acute Therapy ◽  
Vasopressor Support ◽  
Distributive Shock

AbstractSystemic capillary leak syndrome (SCLS) is a potentially life-threatening disorder characterized by distributive shock, hypoalbuminemia, and hemoconcentration. It is exceedingly rare in children with less than 20 cases reported to date. The underlying cause for this syndrome remains largely unknown and acute treatment has remained mainly supportive. Prophylaxis with intravenous immunoglobulin (IVIG) has been shown to successfully prevent further episodes in both adults and children. We present a case of a 2-year-old previously healthy male admitted to the pediatric intensive care unit with a clinical course consistent with SCLS. His shock was refractory to aggressive fluid and vasopressor support. Reversal of SCLS with IVIG given in the acute phase had been described in three adult subjects, and for this reason, the decision was made to administer IVIG. Within an hour of administration, hemodynamics stabilized and vasopressor support could be weaned. He has had no further episodes on prophylactic infusions of IVIG. Although the exact mechanism of IVIG in SCLS is unknown, it has proven to be an effective and safe prophylactic therapy, and in our patient, it drastically reversed the acute capillary leak. We suggest that IVIG should be considered as acute therapy in pediatric patients with refractory shock and a clinical course suggestive of SCLS.

scholarly journals Hyperviscosity-related splenic infarction, gastrointestinal ischaemia–reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson’s syndrome)

2020 ◽  
Vol 13 (1) ◽  
pp. e232500
Author(s):  
Jelle Alexander van Erven ◽  
Jolanda Schrama ◽  
Daan Albert Robertus Castelijn
Keyword(s):  
Splenic Infarction ◽  
Capillary Leak Syndrome ◽  
Polycythaemia Vera ◽  
Capillary Leak ◽  
Myeloproliferative Disease ◽  
Systemic Capillary Leak Syndrome ◽  
Ischaemia Reperfusion Injury ◽  
Correct Treatment ◽  
Ischaemia Reperfusion ◽  
Distributive Shock

Clarkson’s syndrome, also known as idiopathic systemic capillary leak syndrome, is characterised by vascular hyperpermeability resulting in intravascular hypovolaemia and shock. A clinician should consider the diagnosis if other causes of shock, for example, sepsis and anaphylaxis, are ruled out and concomitant hyperviscosity is not caused by a myeloproliferative disease. Here, we describe a patient presenting with severe plasma leakage and assumable blood hyperviscosity leading to splenic infarction, gastrointestinal ischaemia–reperfusion syndrome and transient dysarthria. Our patient was first suspected of polycythaemia vera and phlebotomies were performed. Awareness of this syndrome and subsequent correct treatment is essential to prevent complications and to reduce mortality. As in our patient, most patients with Clarkson’s syndrome have a monoclonal gammopathy, light-chain-type kappa. Prophylactic treatment with intravenous immunoglobulin (IVIg) is advised to prevent recurrence of capillary leak. Our patient did not suffer from another symptomatic episode after starting IVIg.

scholarly journals Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

2013 ◽  
Vol 2013 (apr09 1) ◽  
pp. bcr2013009048-bcr2013009048 ◽  
Author(s):  
J. R. Sheehan ◽  
L. Keating ◽  
A. Chan ◽  
A. Walden
Keyword(s):  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Distributive Shock

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.

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