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uremic hemolytic syndrome

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https://www.readbyqxmd.com/read/28078430/blood-urea-nitrogen-to-serum-creatinine-ratio-is-an-accurate-predictor-of-outcome-in-diarrhea-associated-hemolytic-uremic-syndrome-a-preliminary-study
#1
Werner Keenswijk, Jill Vanmassenhove, Ann Raes, Evelyn Dhont, Johan Vande Walle
: Diarrhea-associated hemolytic uremic syndrome (D+HUS) is a common thrombotic microangiopathy during childhood and early identification of parameters predicting poor outcome could enable timely intervention. This study aims to establish the accuracy of BUN-to-serum creatinine ratio at admission, in addition to other parameters in predicting the clinical course and outcome. Records were searched for children between 1 January 2008 and 1 January 2015 admitted with D+HUS. A complicated course was defined as developing one or more of the following: neurological dysfunction, pancreatitis, cardiac or pulmonary involvement, hemodynamic instability, and hematologic complications while poor outcome was defined by death or development of chronic kidney disease...
January 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28075671/hospitalization-rates-for-intussusception-in-children-aged-0-59%C3%A2-months-from-2009-to-2014-in-italy
#2
Vincenzo Restivo, Claudio Costantino, Fabio Tramuto, Francesco Vitale
The real cause of intussusception is not fully understood and a variety of conditions have been associated with it (Meckel diverticulum, polyps, duplication cysts, parasites, Henoch-Schönlein purpura, cystic fibrosis, hemolytic-uremic syndrome and infectious gastroenteritis). Furthermore few European countries, following WHO recommendation to monitor baseline incidence of intussusception before implementation of immunization program for rotavirus, used intussusception rate as a baseline value to compare the same figures in the period before and after introduction of vaccination...
January 11, 2017: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/28074307/clinical-characteristics-and-long-term-outcome-of-diarrhea-associated-hemolytic-uremic-syndrome-a-single-center-experience
#3
Takeshi Ninchoji, Kandai Nozu, Keita Nakanishi, Tomoko Horinouchi, Junya Fujimura, Tomohiko Yamamura, Shogo Minamikawa, Shingo Ishimori, Koichi Nakanishi, Norishige Yoshikawa, Ichiro Morioka, Hiroshi Kaito, Kazumoto Iijima
OBJECTIVES: To clarify the clinical characteristics and long-term outcomes of patients with diarrhea-associated hemolytic uremic syndrome (D + HUS) with a particular focus on time course. METHODS: We retrospectively analyzed the medical records of 61 patients with D + HUS who were admitted to Kobe University Hospital between 1995 and 2015. The onset of D + HUS was defined as day 1 of diarrhea. RESULTS: The age of onset was 4.1 (1...
January 10, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28062285/a-review-on-strategies-for-decreasing-e-%C3%A2-coli-o157-h7-risk-in-animals
#4
REVIEW
Pardis Saeedi, Maryam Yazdanparast, Elham Behzadi, Ali Hatef Salmanian, Seyed Latif Mousavi, Shahram Nazarian, Jafar Amani
Enterohemorrhagic Escherichia coli (EHEC) serotype O157:H7 is a food-borne pathogen that younger children are most prone to this microorganism. Hemolytic Uremic Syndrome (HUS) caused by EHEC, leads to the destruction of red blood cells and kidney failure. The virulence of E.coli O157:H7 is attributed to fimbriae, that facilitate colonization of bacteria within the colon and verotoxins (VT) or Shiga toxins (Stx) that are released into the blood. Although, in most cases, the infection is self-limitedin young children and aged population, it may cause HUS...
January 3, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#5
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/28057640/murine-systemic-thrombophilia-and-hemolytic-uremic-syndrome-from-a-factor-h-point-mutation
#6
Yoshiyasu Ueda, Imran Mohammed, Delu Song, Damodar Gullipalli, Lin Zhou, Sayaka Sato, Yuan Wang, Shuchi Gupta, Zhongjian Cheng, Hong Wang, Jialing Bao, Yingying Mao, Lawrence Brass, X Long Zheng, Takashi Miwa, Matthew Palmer, Joshua Dunaief, Wen-Chao Song
Complement plays a key role in host defense but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure in patients. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28057514/systemic-effects-of-subtilase-cytotoxin-produced-by-escherichia-coli-o113-h21
#7
E Abril Seyahian, Gisela Oltra, Federico Ochoa, Santiago Melendi, Ricardo Hermes, James C Paton, Adrienne W Paton, Nestor Lago, Mauricio Castro Parodi, Alicia Damiano, Cristina Ibarra, Elsa Zotta
: Subtilase cytotoxin (SubAB) is a member of the AB5 cytotoxin family and is produced by certain strains of Shiga toxigenic Escherichia coli. The toxin is known to be lethal to mice, but the pathological mechanisms that contribute to Uremic Hemolytic Syndrome (HUS) are poorly understood. In this study we show that intraperitoneal injection of a sublethal dose of SubAB in rats triggers a systemic response, with ascitic fluid accumulation, heart hypertrophy and damage to the liver, colon and kidney...
January 2, 2017: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/28056875/turkish-pediatric-atypical-hemolytic-uremic-syndrome-registry-initial-analysis-of-146-patients
#8
Nesrin Besbas, Bora Gulhan, Oguz Soylemezoglu, Z Birsin Ozcakar, Emine Korkmaz, Mutlu Hayran, Fatih Ozaltin
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management. METHODS: To identify the characteristics of aHUS in Turkish children, an industry-independent registry was established for data collection that includes both retrospective and prospective patients. RESULTS: In total, 146 patients (62 boys, 84 girls) were enrolled; 53 patients (36...
January 5, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28056011/escherichia-coli-o157-h7-infections-associated-with-contaminated-pork-products-alberta-canada-july-october-2014
#9
Lance Honish, Nisha Punja, Sarah Nunn, Dale Nelson, Nyall Hislop, Garth Gosselin, Natisha Stashko, Darlene Dittrich
During July-October 2014, an outbreak of 119 Escherichia coli O157:H7 infections in Alberta, Canada was identified through notifiable disease surveillance and investigated by local, provincial, and federal public health and food regulatory agencies. Twenty-three (19%) patients were hospitalized, six of whom developed hemolytic uremic syndrome; no deaths were reported. Informed by case interviews, seven potential food sources were identified and investigated. The majority of patients reported having consumed meals containing pork at Asian-style restaurants in multiple geographically diverse Alberta cities during their exposure period...
January 6, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/28042026/dexamethasone-prevents-motor-deficits-and-neurovascular-damage-produced-by-shiga-toxin-2-and-lipopolysaccharide-in-the-mouse-striatum
#10
Alipio Pinto, Adriana Cangelosi, Patricia Geoghegan, Jorge Goldstein
Shiga toxin 2 (Stx2) from enterohemorrhagic Escherichia coli (EHEC) causes bloody diarrhea and Hemolytic Uremic Syndrome (HUS) that may derive to fatal neurological outcomes. Neurological abnormalities in the striatum are frequently observed in affected patients and in studies with animal models while motor disorders are usually associated with pyramidal and extra pyramidal systems. A translational murine model of encephalopathy was employed to demonstrate that systemic administration of a sublethal dose of Stx2 damaged the striatal microvasculature and astrocytes, increase the blood brain barrier permeability and caused neuronal degeneration...
December 30, 2016: Neuroscience
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#11
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28025630/adjustment-of-eculizumab-dosage-pattern-in-patients-with-atypical-hemolytic-uremic-syndrome-with-suboptimal-response-to-standard-treatment-pattern
#12
Camino García Monteavaro, Carmen Peralta Roselló, Borja Quiroga, José María Baltar Martín, Lorena Castillo Eraso, Fernando de Álvaro Moreno, Alberto Martínez Vea, María Teresa Visus-Fernández de Manzanos
In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14 ± 2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27989426/surgical-management-of-complicated-necrotizing-pneumonia-in-children
#13
Jin-Yao Lai, Wendy Yang, Yung-Ching Ming
BACKGROUND: There are no well-established indications for the surgical management of acute necrotizing pneumonitis in children. This study presents our experience regarding this challenging topic. METHODS: Between 2002 and 2009, 56 necrotizing pneumonitis patients with empyema were treated surgically. The outcomes were analyzed retrospectively. Computed tomography findings of massive lung necrosis or large cavities involving more than 50% of the involved lobe were deemed to be complicated necrotizing pneumonitis...
October 28, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/27989322/atypical-hemolytic-uremic-syndrome-and-c3-glomerulopathy-conclusions-from-a-kidney-disease-improving-global-outcomes-kdigo-controversies-conference
#14
Timothy H J Goodship, H Terence Cook, Fadi Fakhouri, Fernando C Fervenza, Véronique Frémeaux-Bacchi, David Kavanagh, Carla M Nester, Marina Noris, Matthew C Pickering, Santiago Rodríguez de Córdoba, Lubka T Roumenina, Sanjeev Sethi, Richard J H Smith
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options...
December 15, 2016: Kidney International
https://www.readbyqxmd.com/read/27986662/a-pathogenic-role-of-complement-in-arterial-hypertension-and-hypertensive-end-organ-damage
#15
Ulrich O Wenzel, Marlies Bode, Jörg Köhl, Heimo Ehmke
The self-amplifying cascade of messenger and effector molecules of the complement system serves as a powerful danger sensing system that protects the host from a hostile microbial environment, while maintaining proper tissue and organ function through effective clearance of altered or dying cells. As an important effector arm of innate immunity it plays also important roles in the regulation of adaptive immunity. Innate and adaptive immune response have been identified as crucial players in the pathogenesis of arterial hypertension and hypertensive end organ damage...
December 16, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27982687/angiotensinogen-and-interleukin-18-as-markers-of-chronic-kidney-damage-in-children-with-a-history-of-hemolytic-uremic-syndrome
#16
K Lipiec, P Adamczyk, E Świętochowska, K Ziora, M Szczepańska
Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). It is clinically important to investigate the markers of a poor prognosis. The levels of angiotensinogen (AGT) and interleukin-18 (IL-18) in serum and urine were evaluated. Study was conducted in 29 children with a history of HUS. Serum and urine AGT concentration was significantly higher in children after HUS as compared to the control group. No differences depending on the type of HUS and gender were noted...
December 16, 2016: Physiological Research
https://www.readbyqxmd.com/read/27978506/a-minireview-on-the-in-vitro-and-in-vivo-experiments-with-anti-escherichia-coli-o157-h7-phages-as-potential-biocontrol-and-phage-therapy-agents
#17
REVIEW
Salehe Sabouri, Zargham Sepehrizadeh, Sahar Amirpour-Rostami, Mikael Skurnik
Phage therapy is an old method of combating bacterial pathogens that has recently been taken into consideration due to the alarming spread of antibiotic resistance. Escherichia coli O157:H7 is a foodborne pathogen that causes hemorrhagic colitis and life-threatening Hemolytic Uremic Syndrome (HUS). There are several studies on isolation of specific phages against E. coli O157:H7 and more than 60 specific phages have been published so far. Although in vitro experiments have been successful in elimination or reduction of E...
December 11, 2016: International Journal of Food Microbiology
https://www.readbyqxmd.com/read/27977428/breaking-down-the-complement-system-a-review-and-update-on-novel-therapies
#18
Yuvaram N V Reddy, Andrew M Siedlecki, Jean M Francis
PURPOSE OF REVIEW: The complement system represents one of the more primitive forms of innate immunity. It has increasingly been found to contribute to pathologies in the native and transplanted kidney. We provide a concise review of the physiology of the complement cascade, and discuss current and upcoming complement-based therapies. RECENT FINDINGS: Current agents in clinical use either bind to complement components directly or prevent complement from binding to antibodies affixed to the endothelial surface...
December 12, 2016: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/27976568/an-unexpected-diagnostic-course-of-systemic-lupus-erythematosus
#19
Aysel Taktak, Adem Köksoy, Özge Başaran, Saba Kiremitçi, Banu Acar, Nermin Uncu, Nilgün Çakar
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27974740/a-case-report-and-literature-review-of-eculizumab-withdrawal-in-atypical-hemolytic-uremic-syndrome
#20
Borja Quiroga, Alberto de Lorenzo, Cristina Vega, Fernando de Alvaro
BACKGROUND Recent advances in the treatment of atypical hemolytic-uremic syndrome (aHUS) have resulted to better long-term survival rates for patients with this life-threatening disease. However, many questions remain such as whether or not long-term treatment is necessary in some patients and what are the risks of prolonged therapy. CASE REPORT Here, we discuss the case of a 37-year-old woman with CFH and CD46 genetic abnormalities who developed aHUS with severe renal failure. She was successfully treated with three doses of rituximab and a three month treatment with eculizumab...
December 15, 2016: American Journal of Case Reports
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