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https://www.readbyqxmd.com/read/29894349/anesthetic-considerations-for-a-patient-with-chronic-systemic-capillary-leak-syndrome-a-case-report
#1
Resham Datta, Jason K Panchamia
Systemic capillary leak syndrome (SCLS) is an idiopathic vascular hyperpermeability that leads to profound hypotension, hypoalbuminemia, and hemoconcentration. Sixty years since its inauguration into literature, SCLS is still elusive, underdiagnosed, and profoundly dangerous. Few authors have documented anesthetic implications of classical acute SCLS, a form with complete recovery between exacerbations. However, minimal information is available about chronic SCLS, continuous generalized edema with or without exacerbations...
June 11, 2018: A&A practice
https://www.readbyqxmd.com/read/29892202/clinical-and-epidemiologic-profile-and-predictors-of-outcome-of-poisonous-snake-bites-an-analysis-of-1-500-cases-from-a-tertiary-care-center-in-malabar-north-kerala-india
#2
Kg Sajeeth Kumar, Santhosh Narayanan, V Udayabhaskaran, N K Thulaseedharan
Background: Snake bites are a neglected public health problem in all tropical countries, and particularly in Malabar region of Kerala, India. Demographic characteristics and clinical parameters vary in this part of the world, and studies based on them are scarce. Increased incidence of capillary leak syndrome (CLS) in hemotoxic snake bites and emergence of cases of hump-nosed pit viper (HNPV, Hypnale hypnale ) envenomation are some notable features seen here. Objectives: The study was conducted to assess the epidemiological profile and clinical features to identify factors contributing to outcome in patients with venomous snakebites...
2018: International Journal of General Medicine
https://www.readbyqxmd.com/read/29879013/capillary-leak-syndrome-and-aseptic-meningitis-in-a-patient-with-kawasaki-disease-a-case-report
#3
Yufeng Zhang, Han Wan, Maosheng Du, Huiling Deng, Jia Fu, Yu Zhang, Xiaoyan Wang, Ruiqing Liu
RATIONALE: Kawasaki disease (KD) is an acute vasculitis of childhood, coronary complications are the most serious and classic complications of this disease. However, simultaneous complications such as systemic capillary leak syndrome (CLS) and aseptic meningitis are rarely reported. PATIENT CONCERNS: A 19-month-old boy had continuous fever for 6 days, rash for 3 days, and somnolence for 1 day. DIAGNOSES: The boy was diagnosed with KD presenting with SCLS and aseptic meningitis...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29779703/idiopathic-systemic-capillary-leak-syndrome-presenting-as-septic-shock-a-case-report
#4
Eamon P Raith, Joshua F Ihle, Jennifer Jamieson, Anna Kalff, Julian Bosco
BACKGROUND: Idiopathic capillary leak syndrome (Clarkson's Disease) is a rare angiopathy with a heterogenous phenotype that may present as distributive shock refractory to resuscitative management. OBJECTIVE: We report a case of idiopathic systemic capillary leak syndrome presenting as septic shock. METHODS: Structured case report and review of the literature. RESULTS: A 27-year old man admitted to our institution with coryzal symptoms rapidly deteriorated with presumed sepsis, leading to intensive care unit admission...
May 17, 2018: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/29777280/primary-draining-vein-stenting-for-obstructive-total-anomalous-pulmonary-venous-connection-in-neonates-with-right-atrial-isomerism-and-functional-single-ventricle-improves-outcome
#5
Masataka Kitano, Takaya Hoashi, Takashi Kakuta, Kazuto Fujimoto, Akira Miyake, Ken-Ichi Kurosaki, Hazime Ichikawa, Isao Shiraishi
For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVCR group) was 30% (3/10). Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29732372/the-role-of-failing-autonomic-nervous-system-on-life-threatening-idiopathic-systemic-capillary-leak-syndrome
#6
Riccardo Colombo, Maddalena Alessandra Wu, Emanuele Catena, Andrea Perotti, Tommaso Fossali, Federico Cioffi, Roberto Rech, Antonio Castelli, Marco Cicardi
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease that involves the endothelium and microcirculation, leading to an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. The consequence of the capillary leakage is a life-threatening hypovolemic shock that can lead to lethal multiple organ dysfunction. The autonomic nervous system (ANS) is central in regulating the cardiovascular response to hypovolemia, but ANS modulation in ISCLS has not yet been investigated...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29720802/a-viral-polymyositis-masquerade-life-threatening-case-of-juvenile-dermatomyositis-complicated-by-systemic-capillary-leak-syndrome
#7
Bhaskara P Shelley, Shrijeet Chakraborti
This is a case report of an 8-year-old boy who developed an atypical, rare subphenotype of autoimmune inflammatory acute juvenile dermatomyositis (JDM), initially masquerading as viral polymyositis (PM)-like presentation, that was complicated by a hitherto unreported fulminant, life-threatening pediatric systemic capillary leak syndrome (SCLS). We highlight the close differential between viral PM and JDM, the baffling clinical syndromic constellation of hypotension with hemoconcentration - a "shock"-like syndrome, hypoalbuminemia without albuminuria, and generalized edema with the atypical JDM presentation, and stress crucial need to implement early aggressive, multipronged immunomodulatory treatment along with intensive fluid resuscitation which saved the life, this patient from a stormy, and turbulent 4-week clinical illness...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29706650/innovative-strategies-minimize-engraftment-syndrome-in-multiple-myeloma-patients-with-novel-induction-therapy-following-autologous-hematopoietic-stem-cell-transplantation
#8
Gonzalo Gutiérrez-García, Montserrat Rovira, Laura Magnano, Laura Rosiñol, Alex Bataller, María Suárez-Lledó, María Teresa Cibeira, Carlos Fernández de Larrea, Marta Garrote, Sofia Jorge, Ana Moreno, Luis Gerardo Rodríguez-Lobato, Enric Carreras, Maribel Díaz-Ricart, Marta Palomo, Carmen Martínez, Alvaro Urbano-Ispizua, Joan Bladé, Francesc Fernández-Avilés
Autologous stem cell transplantation (PBSCT) is standard for young patients in MM and its TRM has decreased after the 2000s. Bortezomib and immunomodulatory agents (IMiDs) in MM have improved the outcome. However, they seem to boost pro-inflammatory stage increasing the incidence of engraftment syndrome (ES). Favorable factors in PBSCT such as G-CSF could increase inflammatory stage during transplant. Corticosteroids have shown an excellent response of ES and some authors propose them as prophylaxis for ES...
April 29, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29682370/fatal-pneumococcus-sepsis-after-treatment-of-late-antibody-mediated-kidney-graft-rejection
#9
Gunilla Einecke, Jan Hinrich Bräsen, Nils Hanke, Hermann Haller, Anke Schwarz
Antibody-mediated rejection (ABMR) is a major cause of late renal allograft dysfunction and graft loss. Risks and benefits of treatment of late ABMR have not been evaluated in randomized clinical trials. We report on a 35-year-old patient with deterioration in renal function and progressive proteinuria 15 years after transplantation. Recurrent infections after a splenectomy following traumatic splenic rupture 3 years earlier had led to reduction of immunosuppression. Renal transplant biopsy showed glomerular double contours, 40% fibrosis/tubular atrophy, peritubular capillaritis, and positive C4d staining indicating chronic-active ABMR...
2018: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29657372/targeted-interventions-in-critically-ill-children-with-severe-dengue
#10
Suchitra Ranjit, Gokul Ramanathan, Balasubramaniam Ramakrishnan, Niranjan Kissoon
Background: The World Health Organization guidelines provide suggestions on early recognition and treatment of severe dengue (SD); however, mortality in this group can be high and is related both to disease severity and the treatment complications. Subjects and Methods: In this prospective observational study, we report our results where standard therapy (ST) was enhanced by Intensive Care Unit (ICU) supportive measures that have proven beneficial in other conditions that share similar pathophysiology of capillary leak and fluid overload...
March 2018: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29619573/-idiopathic-systemic-capillary-leak-syndrome-clarkson-disease-a-rare-cause-of-recurrent-life-threatening-edema
#11
REVIEW
S Dasdelen, S-O Grebe
This case report describes the episodic occurrence of severe generalized edema in a young female patient, who developed hypertension with a massive hemoconcentration (hematocrit >0.5, hemoglobin >20g/dl) and hypoalbuminemia during the course of these acute disease phases. After the first two disease exacerbations were overcome, there was a complete regression of symptoms. After a long symptom-free interval, a new exacerbation occurred as a result of which critical organ ischemia occurred due to the severe hypotension and massive edema...
April 4, 2018: Der Internist
https://www.readbyqxmd.com/read/29584906/capillary-leak-syndrome-in-daboia-russelii-bite-a-complication-associated-with-poor-outcome
#12
Pradip P Kendre, Manoj P Jose, Abraham M Varghese, Jaideep C Menon, Joseph K Joseph
Background: Capillary leak syndrome (CLS) has been previously observed as a complication of Daboia russelii bite but not clearly defined or studied in length. This observational case-control study evaluates the mortality along with associated clinical and laboratory features. Methods: Twenty-five patients who developed CLS were compared with 25 patients without CLS following Daboia russelii (Russell's viper) bite. Results: Development of CLS is associated with a significantly high risk of mortality; 11 (44%) patients with CLS died compared with 1 (4%) control (odds ratio 18...
February 1, 2018: Transactions of the Royal Society of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29564922/recognition-and-management-of-idiopathic-systemic-capillary-leak-syndrome-an-evidence-based-review
#13
Noor Ul-Ain Baloch, Marvi Bikak, Abdul Rehman, Omar Rahman
INTRODUCTION: Idiopathic systemic capillary leak syndrome (SCLS) is a unique disorder characterized by episodes of massive systemic leak of intravascular fluid leading to volume depletion and shock. A typical attack of SCLS consists of prodromal, leak and post-leak phases. Complications, such as compartment syndrome and pulmonary edema, usually develop during the leak and post-leak phases respectively. Judicious intravenous hydration and early use of vasopressors is the cornerstone of management in such cases...
May 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29559757/successful-use-of-extracorporeal-life-support-in-a-hematopoietic-stem-cell-transplant-patient-with-neuroblastoma
#14
Feifei Z Williams, Atul Vats, Thomas Cash, James D Fortenberry
Respiratory failure associated with hematopoietic stem cell transplantation (HSCT) has been considered a contraindication for use of extracorporeal membrane oxygenation (ECMO) at many centers. We describe a child with neuroblastoma and hypoxemic respiratory failure following HSCT who was successfully managed with veno-venous (VV) ECMO. The patient was an 18-month-old female with high-risk neuroblastoma status post tumor resection, chemotherapy, autologous HSCT, and primary site radiation. On day 113 posttransplant while receiving maintenance immunotherapy, she had an acute respiratory decompensation because of rhinovirus, aspiration pneumonia, and capillary leak syndrome...
March 2018: Journal of Extra-corporeal Technology
https://www.readbyqxmd.com/read/29501285/impact-and-safety-of-open-lung-biopsy-in-patients-with-acute-respiratory-distress-syndrome-ards
#15
G Ortiz, M Garay, D Mendoza, P Cardinal-Fernández
INTRODUCTION: Acute respiratory distress syndrome (ARDS) is an inflammatory lung disorder, and its pathological hallmark is diffuse alveolar damage (DAD). Given that open lung biopsy (OLB) can sometimes result in severe side effects, it is rarely performed in patients with ARDS. AIM: The aims of this study were to describe: (a) the rate of treatment change associated with the histological result; and (b) the incidence of side effects induced by OLB. DESIGN AND PATIENTS: A retrospective, single-center, descriptive observational study was carried out in Hospital Santa Clara (Bogotá, Colombia) from February 2007 to January 2014...
February 28, 2018: Medicina Intensiva
https://www.readbyqxmd.com/read/29449526/raised-serum-levels-of-syndecan-1-cd138-in-a-case-of-acute-idiopathic-systemic-capillary-leak-syndrome-scls-clarkson-s-disease
#16
Ole Wilhelm Bøe, Kjell Sveen, Magne Børset, Kirk M Druey
BACKGROUND Systemic capillary leak syndrome (SCLS) (Clarkson's disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80-90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown...
February 16, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29402721/optimized-administration-of-hetil-15-expands-lymphocytes-and-minimizes-toxicity-in-rhesus-macaques
#17
Cristina Bergamaschi, Dionysios C Watson, Antonio Valentin, Jenifer Bear, Cody J Peer, William D Figg, Barbara K Felber, George N Pavlakis
The common γ-chain cytokine interleukin-15 (IL-15) plays a significant role in regulating innate and adaptive lymphocyte homeostasis and can stimulate anti-tumor activity of leukocytes. We have previously shown that the circulating IL-15 in the plasma is the heterodimeric form (hetIL-15), produced upon co-expression of IL-15 and IL-15 Receptor alpha (IL-15Rα) polypeptides in the same cell, heterodimerization of the two chains and secretion. We investigated the pharmacokinetic and pharmacodynamic profile and toxicity of purified human hetIL-15 cytokine upon injection in rhesus macaques...
February 2, 2018: Cytokine
https://www.readbyqxmd.com/read/29364440/how-relevant-are-vascular-endothelial-growth-factor-and-intercellular-adhesion-molecule-in-the-systemic-capillary-leak-syndrome-of-psoriasis
#18
REVIEW
Aline Lopes Bressan, Daniele Pereira, Paula Mota Medeiros, Sueli Carneiro, Luna Azulay-Abulafia
Psoriasis is a chronic disease, characterized by erythematous scaly lesions, presented in eight different forms: plaques, guttate, pustular, erythrodermic, inverse, nail and scalp psoriasis, and psoriatic arthritis. Its development depends on genetic factors, external stimulus and immune response alteration.1 Proinflammatory cytokines such as TNF-alpha, IL-12 and 23 may also be involved. In the worst cases, systemic complications linked to endothelial alterations may occur. A literature review was conducted for a better understanding of what roles VEGF (vascular endothelial growth factor) and ICAM-1 (intercellular adhesion molecule) have, among other cytokines, in systemic capillary leak syndrome, involved in erythrodermic and pustular psoriasis, the most unstable forms of the disease...
November 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29360169/adrenomedullin-surges-are-linked-to-acute-episodes-of-the-systemic-capillary-leak-syndrome-clarkson-disease
#19
Zhihui Xie, Wei-Sheng Chen, Yuzhi Yin, Eunice C Chan, Kaoru Terai, Lauren M Long, Timothy G Myers, Arkadiusz Z Dudek, Kirk M Druey
BACKGROUND: Systemic Capillary Leak Syndrome (SCLS) is an extremely rare and life-threatening vascular disorder of unknown etiology. SCLS is characterized by abrupt and transient episodes of hypotensive shock and edema due to plasma leakage into peripheral tissues. The disorder has garnered attention recently because its initial presentation resembles more common vascular disorders including systemic anaphylaxis, sepsis, and acute infections with the Ebola/Marburg family of filoviruses...
April 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29360075/-hemophagocytosis-secondary-to-dengue-fever
#20
María L Rosenberg, Gonzalo L Echavarría, Ana V Ludueña, Gabriela Estrada, María M Molina
Dengue virus infection constitutes a major public health problem worldwide. It is caused by a virus belonging to the Flaviviridae family. It produces a wide range of clinical presentations, from asymptomatic infection to severe forms of the disease with hemorrhagic fever or shock secondary to capillary leak syndrome. Four serotypes have been described; serotype 2 and serotype 3 are associated with the most severe forms of the disease. The diagnosis is based on laboratory tests aimed to detect antibodies, viral RNA, or antigens in serum...
2018: Medicina
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