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https://www.readbyqxmd.com/read/29222940/an-open-conformation-of-adamts13-is-a-hallmark-of-acute-acquired-thrombotic-thrombocytopenic-purpura
#1
E Roose, A S Schelpe, B S Joly, M Peetermans, P Verhamme, J Voorberg, A Greinacher, H Deckmyn, S F De Meyer, P Coppo, A Veyradier, K Vanhoorelbeke
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is an autoimmune disorder characterized by absent ADAMTS13 activity and presence of anti-ADAMTS13 autoantibodies. Recently it was shown that ADAMTS13 adopts a folded or an open conformation. OBJECTIVES: As conformational changes in self-antigens play a role in the pathophysiology of different autoimmune diseases, we hypothesized that the conformation of ADAMTS13 changes during acute aTTP. METHODS: Antibodies recognizing cryptic epitopes in the spacer domain were generated...
December 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29222317/thrombocytopenia-in-hospitalized-patients-approach-to-the-patient-with-thrombotic-microangiopathy
#2
REVIEW
Marie Scully
Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29216383/genetic-susceptibility-to-hemolytic-uremic-syndrome-after-shiga-toxin-producing-escherichia-coli-stec-infection-a-centers-for-disease-control-and-prevention-cdc-foodnet-study
#3
Asha R Kallianpur, Yuki Bradford, Rajal K Mody, Katie N Garman, Nicole Comstock, Sarah L Lathrop, Carol Lyons, Amy Saupe, Katie Wymore, Jeffrey A Canter, Lana M Olson, Amanda Palmer, Timothy F Jones
Background: Post-diarrheal hemolytic-uremic syndrome (D+HUS) following Shiga toxin-producing Escherichia coli (STEC) infection is a serious condition lacking specific treatment. Host immune dysregulation and genetic susceptibility to complement hyperactivation are implicated in non-STEC-related HUS. However, genetic susceptibility to D+HUS remains largely uncharacterized. Methods: Patients with culture-confirmed STEC diarrhea, identified through the CDC FoodNet surveillance system (2007-2012), were serotyped and classified by laboratory and/or clinical criteria as suspected, probable, or confirmed D+HUS, or as controls and genotyped at 200 loci linked to non-diarrheal HUS or similar pathologies...
December 6, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29215086/genetic-predisposition-to-infection-in-a-case-of-atypical-hemolytic-uremic-syndrome
#4
Lambertus van den Heuvel, Kristian Riesbeck, Omaima El Tahir, Valentina Gracchi, Mariann Kremlitzka, Servaas A Morré, A Marceline van Furth, Birendra Singh, Marcin Okrój, Nicole van de Kar, Anna M Blom, Elena Volokhina
Most cases of hemolytic uremic syndrome (HUS) are caused by infection with enterohemorrhagic Escherichia coli (EHEC). Genetic defects causing uncontrolled complement activation are associated with the more severe atypical HUS (aHUS). Non-EHEC infections can trigger the disease, however, complement defects predisposing to such infections have not yet been studied. We describe a 2-month-old patient infected with different Gram-negative bacterial species resulting in aHUS. Serum analysis revealed slow complement activation kinetics...
November 13, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/29214442/shiga-toxin-triggers-endothelial-and-podocyte-injury-the-role-of-complement-activation
#5
REVIEW
Carlamaria Zoja, Simona Buelli, Marina Morigi
Shiga toxin (Stx)-producing Escherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF-κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, culminating in leukocyte adhesion and platelet thrombus formation on the activated endothelium...
December 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29183554/molecular-characterisation-of-human-shiga-toxin-producing-escherichia-coli-o26-strains-results-of-an-outbreak-investigation-romania-february-to-august-2016
#6
Codruţa-Romaniţa Usein, Adriana Simona Ciontea, Cornelia Mãdãlina Militaru, Maria Condei, Sorin Dinu, Mihaela Oprea, Daniela Cristea, Valeria Michelacci, Gaia Scavia, Lavinia Cipriana Zota, Alina Zaharia, Stefano Morabito
IntroductionAt the beginning of 2016, an increase in paediatric haemolytic uremic syndrome (HUS) cases was observed in Romania. The microbiological investigations allowed isolation of Shiga toxin-producing Escherichia coli (STEC) O26 as the causative agent from most cases. Methods: An enhanced national surveillance of HUS and severe diarrhoea was established across the country following the identification of the first cases and was carried out until August 2016. A total of 15 strains were isolated from 10 HUS and five diarrhoea cases...
November 2017: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/29178486/developing-and-characterizing-mr-ct-visible-materials-used-in-qa-phantoms-for-mrgrt-systems
#7
Angela Steinmann, R Jason Stafford, Gabriel Sawakuchi, Zhifei Wen, Laurence Court, Clifton D Fuller, David Followill
PURPOSE: Synthetic tissue equivalent (STE) materials currently used to simulate tumor and surrounding tissues for IROC-Houston's anthropomorphic head and thorax QA phantoms cannot be visualized using magnetic resonance (MR) imaging. The purpose of this study was to characterize dual MR/CT visible STE materials that can be used in an end-to-end QA phantom for MR guided radiotherapy (MRgRT) modalities. METHODS: Over 80 materials' MR, CT, and dosimetric STE properties were investigated for use in MRgRT QA phantoms...
November 27, 2017: Medical Physics
https://www.readbyqxmd.com/read/29169004/idiopathic-atypical-haemolytic-uraemic-syndrome-presenting-with-acute-dystonia
#8
M Rizwan, K E Maduemem
Hemolytic Uremic Syndrome (HUS), a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The atypical HUS (aHUS) results from over activation of complement system with formation of micro thrombi and damage to endothelial cells resulting in renal impairment in 50 % and death in 25 %, commonly in untreated patients. We report an intriguing case of aHUS presenting with acute onset of movement disorder and fluctuating delirium.
August 12, 2017: Irish Medical Journal
https://www.readbyqxmd.com/read/29167171/advances-in-our-understanding-of-the-pathogenesis-of-hemolytic-uremic-syndromes
#9
Emily Elizabeth Bowen, Richard John Mark Coward
Hemolytic uremic syndrome (HUS) is major global health care issue as it is the leading cause of acute kidney injury in children. It is a triad of acute kidney injury, microangiopathic hemolytic anemia and thrombocytopenia. It is classified as a glomerular thrombotic microangiopathy. In recent years major advances in our understanding of complement driven inherited rare forms of HUS have been achieved. However, 90% of cases of HUS are associated with a Shiga toxin producing enteric pathogen. The precise pathological mechanisms in this setting are yet to be elucidated...
November 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29160119/a-review-of-the-infection-pathogenesis-and-prophylaxis-recommendations-in-patients-with-chronic-lymphocytic-leukemia
#10
Tamar Tadmor, Manfred Welslau, Iwoma Hus
The majority of patients with CLL will suffer from infections during their disease, accounting for approximately 60% of deaths in CLL. Patients are predisposed to infection due to immune defects related to the primary disease, and as a result of therapy. The range of infectious complications has evolved alongside therapeutic advances in the treatment of CLL. More recently several novel therapeutic compounds have been introduced in CLL, whose unique safety profiles will probably have an impact on the prophylaxis and management of infections in these patients...
November 27, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29156596/microvesicle-involvement-in-shiga-toxin-associated-infection
#11
REVIEW
Annie Villysson, Ashmita Tontanahal, Diana Karpman
Shiga toxin is the main virulence factor of enterohemorrhagic Escherichia coli, a non-invasive pathogen that releases virulence factors in the intestine, causing hemorrhagic colitis and, in severe cases, hemolytic uremic syndrome (HUS). HUS manifests with acute renal failure, hemolytic anemia and thrombocytopenia. Shiga toxin induces endothelial cell damage leading to platelet deposition in thrombi within the microvasculature and the development of thrombotic microangiopathy, mostly affecting the kidney. Red blood cells are destroyed in the occlusive capillary lesions...
November 19, 2017: Toxins
https://www.readbyqxmd.com/read/29152837/effect-of-nanoliposomes-containing-zataria-multiflora-boiss-essential-oil-on-gene-expression-of-shiga-toxin-2-in-escherichia-coli-o157-h7
#12
Seyed Amin Khatibi, Ali Misaghi, Mir-Hassan Moosavy, Afshin Akhondzadeh Basti, Samira Mohamadian, Ali Khanjari
AIMS: Enterohaemorrhagic Escherichia coli (EHEC) serotype O157:H7 as a major human pathogen is responsible for food borne outbreaks, bloody diarrhea, hemorrhagic colitis and hemolytic uremic syndrome (HUS) and even death. In this study, the antibacterial activity of the Zataria multiflora essential oil (ZMEO) and nanoliposome-encapsulated ZMEO was evaluated on the pathogenicity of Escherichia coli O157:H7. METHODS AND RESULTS: The minimum inhibitory concentrations (MIC) of essential oil (EO) were determined against the bacterium before and after encapsulation into nanoliposome...
November 20, 2017: Journal of Applied Microbiology
https://www.readbyqxmd.com/read/29152520/a-case-of-escherichia-coli-hemolytic-uremic-syndrome-in-a-10-year-old-male-with-severe-neurologic-involvement-successfully-treated-with-eculizumab
#13
Malia Rasa, James Musgrave, Keith Abe, Len Tanaka, Konstantine Xoinis, Bruce Shiramizu, Gretchen Foskett, Rhiana Lau
Hemolytic uremic syndrome (HUS) can be classified as typical and atypical, and the treatment recommendations currently differ between the 2 types. Eculizumab is recommended as first-line treatment for atypical HUS; however, its use in typical HUS has been controversial. We report a case of a 10-year-old male with severe neurologic impairment who was successfully treated with eculizumab, which was started 4 days after onset of neurologic symptoms. Our case supports the use of eculizumab in typical HUS with neurologic involvement, even when given later in the course, as the pathophysiology of typical HUS has been shown to involve activation of the complement pathway, similar to atypical HUS...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29146406/central-nervous-involvement-by-chronic-lymphocytic-leukaemia
#14
Dariusz Szczepanek, Ewa Wąsik-Szczepanek, Agnieszka Szymczyk, Małgorzata Wach, Maria Cioch, Monika Podhorecka, Ewelina Grywalska, Marek Hus
Inclusion of the central nervous system (CNS) in the course of chronic lymphocytic leukaemia (CLL) is rare. At the moment no risk factors or proven treatment methods are known. The disease is described both in its early phase and during its acceleration period, thus it has been suggested that there might be independent mechanisms influencing the development of this condition. As there are no unified diagnostic procedure algorithms each patient needs to be assessed individually. CLL can manifest mostly in elderly people, for whom a possibility of development of neurological disorders with their aetiology different from leukaemia, should also be taken into consideration...
November 1, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29142942/complement-activation-induces-neutrophil-adhesion-and-neutrophil-platelet-aggregate-formation-on-vascular-endothelial-cells
#15
Magdalena Riedl, Damien G Noone, Meraj A Khan, Fred G Pluthero, Walter H A Kahr, Nades Palaniyar, Christoph Licht
Introduction: Atypical hemolytic uremic syndrome is a thrombotic microangiopathy, which is linked to hereditary or autoimmune defects in complement activators or regulators present in blood and on vascular endothelial cells. Acute thrombotic microangiopathy episodes are typically preceded by infections, which by themselves would not be expected to manifest HUS. Thus, it is possible that the host immune response contributes to the precipitation of aHUS. However, the mechanisms involved are not fully understood...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29134241/ct-based-evaluation-of-volumetric-bone-density-in-fragility-fractures-of-the-pelvis-a-matched-case-control-analysis
#16
D Schönenberg, R Guggenberger, D Frey, H-C Pape, H-P Simmen, G Osterhoff
This matched case-control study compared the computed tomography (CT)-based regional bone density of patients with fragility fractures of the sacrum to a control without fracture. Patients with a sacral fracture demonstrated a significantly lower regional bone density of the sacrum, the sacral bone density not being correlated with the BMD by DXA of the spine. INTRODUCTION: The aim of this study is to compare the computed tomography-based regional bone density measured by Hounsfield units (HUs) in patients with and without fragility fractures of the sacrum...
November 13, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/29121863/multiplex-real-time-pcr-assay-for-detection-of-escherichia-coli-o157-h7-and-screening-for-non-o157-shiga-toxin-producing-e-coli
#17
Baoguang Li, Huanli Liu, Weimin Wang
BACKGROUND: Shiga toxin-producing Escherichia coli (STEC), including E. coli O157:H7, are responsible for numerous foodborne outbreaks annually worldwide. E. coli O157:H7, as well as pathogenic non-O157:H7 STECs, can cause life-threating complications, such as bloody diarrhea (hemolytic colitis) and hemolytic-uremic syndrome (HUS). Previously, we developed a real-time PCR assay to detect E. coli O157:H7 in foods by targeting a unique putative fimbriae protein Z3276. To extend the detection spectrum of the assay, we report a multiplex real-time PCR assay to specifically detect E...
November 9, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/29114374/prevention-of-chemotherapy-induced-nephrotoxicity-in-children-with-cancer
#18
REVIEW
Fatemeh Ghane Sharbaf, Hamid Farhangi, Farahnak Assadi
Children with cancer treated with cytotoxic drugs are frequently at risk of developing renal dysfunction. The cytotoxic drugs that are widely used for cancer treatment in children are cisplatin (CPL), ifosfamide (IFO), carboplatin, and methotrexate (MTX). Mechanisms of anticancer drug-induced renal disorders are different and include acute kidney injury (AKI), tubulointerstitial disease, vascular damage, hemolytic uremic syndrome (HUS), and intrarenal obstruction. CPL nephrotoxicity is dose-related and is often demonstrated with hypomagnesemia, hypokalemia, and impaired renal function with rising serum creatinine and blood urea nitrogen levels...
2017: International Journal of Preventive Medicine
https://www.readbyqxmd.com/read/29112188/diagnosis-and-treatment-options-of-acquired-hemophilia-a-single-center-experience
#19
Bożena Sokołowska, Justyna Kozińska, Magdalena Kozioł, Ewa Wąsik-Szczepanek, Dariusz Szczepanek, Marek Hus
No abstract text is available yet for this article.
November 6, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29101168/genetically-increased-angiotensin-i-converting-enzyme-alters-peripheral-and-renal-vascular-reactivity-to-angiotensin-ii-and-bradykinin-in-mice
#20
Catherine Chollet, Sandrine Placier, Christos Chatziantoniou, Annette Hus-Citharel, Nathalie Caron, Ronan Roussel, Francois Alhenc-Gelas, Nadine Bouby
Angiotensin 1-converting enzyme (ACE) levels in man are under strong genetic influence. Genetic variation in ACE has been linked to risk for, and progression of, cardiovascular and renal diseases. Causality has been documented in genetically modified mice but mechanisms underlying causality are not completely elucidated. To further document the vascular and renal consequences of a moderate genetic increase in ACE synthesis, we studied genetically modified mice carrying three copies of the ACE gene (3-copy mice) and littermate wild type animals (2-copy mice)...
November 3, 2017: American Journal of Physiology. Heart and Circulatory Physiology
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