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uremic hemolytic syndrome

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https://www.readbyqxmd.com/read/28303771/epidemiology-and-outcome-of-acute-kidney-injury-in-children-a-single-center-study
#1
Werner Keenswijk, Jill Vanmassenhove, Ann Raes, Evelyn Dhont, Johan VandeWalle
BACKGROUND: Information on the epidemiology of Acute Kidney Injury (AKI) in children is scarce. We performed a single center retrospective cohort study to analyze the incidence of AKI, the male/female ratio, the underlying etiology, and age at presentation. We also aimed to assess outcome measured by mortality, duration of PICU stay, and development of Chronic Kidney Disease (CKD). METHODS: Records were searched for children presenting with or developing AKI between 1st January 2008 and 1st January 2015...
March 17, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28296951/in-silico-design-expression-and-purification-of-novel-chimeric-escherichia-coli-o157-h7-ompa-fused-to-ltb-protein-in-escherichia-coli
#2
Aytak Novinrooz, Taghi Zahraei Salehi, Roya Firouzi, Sina Arabshahi, Abdollah Derakhshandeh
E. coli O157:H7, one of the major EHEC serotypes, is capable of developing bloody diarrhea, hemorrhagic colitis (HC), and fatal hemolytic uremic syndrome (HUS) and is accompanied by high annual economic loss worldwide. Due to the increased risk of HC and HUS development following antibiotic therapy, the prevention of infections caused by this pathogen is considered to be one of the most effective ways of avoiding the consequences of this infection. The main aim of the present study was to design, express, and purify a novel chimeric protein to develope human vaccine candidate against E...
2017: PloS One
https://www.readbyqxmd.com/read/28296538/hemolytic-uremic-syndrome-findings-of-post-acute-renal-failure-in-light-and-electron-microscopy
#3
Wiesława Salwa-Żurawska, Jakub Żurawski, Aldona Woźniak, Elżbieta Bortkiewicz, Paweł Burchardt, Przemysław Kwiatkowski, Monika Seget, Piotr Tabaczewski
The blood count test results of six patients (five male adolescents and one female adult) who were diagnosed with the hemolytic-uremic syndrome are presented. Certain diverse lesions and especially, their different intensity, were observed. They were referred to the clinical process and the time from syndrome occurrence to biopsy.
March 15, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28287636/transplant-associated-thrombotic-microangiopathy-opening-pandora-s-box
#4
REVIEW
E Gavriilaki, I Sakellari, A Anagnostopoulos, R A Brodsky
Transplant-associated thrombotic microangiopathy (TA-TMA) is an early complication of hematopoietic cell transplantation (HCT). A high mortality rate is documented in patients who are refractory to calcineurin inhibitor cessation. Estimates of TA-TMA prevalence vary significantly and are higher in allogeneic compared with autologous HCT. Furthermore, our understanding of the pathophysiology that is strongly related to diagnosis and treatment options is limited. Recent evidence has linked TA-TMA with atypical hemolytic uremic syndrome, a disease of excessive activation of the alternative pathway of complement, opening the Pandora's box in treatment options...
March 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28283851/serum-ferritin-as-an-indicator-of-the-development-of-encephalopathy-in-enterohemorrhagic-escherichia-coli-induced-hemolytic-uremic-syndrome
#5
Masaki Shimizu, Natsumi Inoue, Mondo Kuroda, Hitoshi Irabu, Maiko Takakura, Hisashi Kaneda, Naotoshi Sugimoto, Kazuhide Ohta, Akihiro Yachie
OBJECTIVES: To investigate the diagnostic value of serum ferritin levels as a marker of disease activity and the development of encephalopathy in hemolytic uremic syndrome (HUS) induced by enterohemorrhagic Escherichia coli. METHODS: Twenty patients with HUS were studied. Serum ferritin levels were compared with clinical features and serum soluble tumor necrosis factor receptor (sTNFR) I and sTNFRII levels. Serum sTNFRI and sTNFRII levels were quantified by enzyme-linked immunosorbent assays...
March 10, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28275964/reversal-of-threatening-blindness-after-initiation-of-eculizumab-in-purtscher-like-retinopathy-secondary-to-atypical-hemolytic-uremic-syndrome
#6
J E Ramos de Carvalho, R O Schlingemann, M Oranje, F J Bemelman, M J van Schooneveld
Purtscher-like retinopathy, a rare manifestation of systemic thrombotic microangiopathy, is a potentially visually debilitating condition with no effective proven treatment. Distinct pathogenic pathways have been proposed as etiological factors. We revisit the etiology of Purtscher-like retinopathy based on the rapid response and profound visual improvement after initiation of systemic intravenous eculizumab, an inhibitor of the complement cascade, in a patient with Purtscher-like retinopathy secondary to familial atypical hemolytic uremic syndrome (aHUS) due to a mutation in complement factor H...
March 8, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28253847/prevalence-and-antimicrobial-susceptibility-of-escherichia-coli-o157-in-beef-at-butcher-shops-and-restaurants-in-central-ethiopia
#7
Ashenafi Feyisa Beyi, Akafete Teklu Fite, Ephrem Tora, Asdesach Tafese, Tadele Genu, Tamirat Kaba, Tariku Jibat Beyene, Takele Beyene, Mesula Geloye Korsa, Fanos Tadesse, Lieven De Zutter, Bruno Maria Goddeeris, Eric Cox
BACKGROUND: Ethiopia bears the largest burden of foodborne diseases in Africa, and diarrheal diseases are the second leading causes of premature deaths. Enterohemorrhagic Escherichia coli O157 causes an asymptomatic infection to severe diarrhea and/or hemolytic-uremic syndrome in humans. METHODS: A total of 440 beef carcass and in-contact surface swabs from 55 butcher shops and 85 minced beef samples from 40 restaurants in central Ethiopia were collected and examined for the presence of E...
March 3, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/28250608/complements-spurned-our-experience-with-atypical-hemolytic-uremic-syndrome
#8
Vidya S Nagar, Rudrarpan Chaterjee, Ankita Sood, Basavaraj Sajjan, Aniruddha Kaushik, Sameer V Vyahalkar
Atypical hemolytic uremic syndrome (aHUS) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system. It results in significant morbidity and mortality if not diagnosed and treated promptly. It lends itself to myriad renal and extrarenal manifestations, all potentially disabling. Eculizumab, a monoclonal antibody to complement C5 is now the widely accepted norm for treatment. However, in resource-limited settings, plasma exchange if instituted early may be as beneficial...
February 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28244618/post-acute-ischemic-change-and-colon-stricture-in-hemolytic-uremic-syndrome
#9
Akie Kobayashi, Shojiro Watanabe, Kazushi Tsuruga, Etsuro Ito, Hiroshi Tanaka
No abstract text is available yet for this article.
February 28, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28239557/shigella-sonnei-and-hemolytic-uremic-syndrome-a-case-report-and-literature-review
#10
Casey Adams, Aaron Vose, Michael B Edmond, Laurel Lyckholm
Hemolytic uremic syndrome (HUS) is a well-described process that is known to cause severe renal dysfunction, thrombocytopenia, and anemia. HUS is typically associated with toxins (shiga-like and shigella toxin) found in strains of E. coli and Shigella spp [1], [2], [3]. We present a case of a 27 year-old man with jaundice, thrombocytopenia, and renal dysfunction who was found to have HUS in the setting of Shigella sonnei infection. Outside of developing countries, cases of HUS related to S. sonnei are largely unreported...
2017: IDCases
https://www.readbyqxmd.com/read/28230102/identification-of-a-new-virulent-clade-in-enterohemorrhagic-escherichia-coli-o26-h11-h-sequence-type-29
#11
Nozomi Ishijima, Ken-Ichi Lee, Tomomi Kuwahara, Haruyuki Nakayama-Imaohji, Saori Yoneda, Atsushi Iguchi, Yoshitoshi Ogura, Tetsuya Hayashi, Makoto Ohnishi, Sunao Iyoda
Enterohemorrhagic Escherichia coli (EHEC) O26 infections cause severe human diseases such as hemolytic uremic syndrome and encephalopathy, and is the predominant serogroup among non-O157 EHEC in many countries. Shiga toxin (Stx), which consists of two distinct types (Stx1 and Stx2), plays a central role in EHEC pathogenesis. The major stx gene type in EHEC O26 strains is stx1, although isolates with only stx2 have emerged in Japan since 2012 and have been reported in Europe. In this study, we selected 27 EHEC O26 strains isolated in Japan and identified a distinct genetic clade within sequence type (ST) 29, designated ST29C1, that carried only stx2 and had the plasmid gene profile ehxA+/katP-/espP+/etpD-...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28224376/a-case-of-atypical-hemolytic-uremic-syndrome-in-a-second-renal-transplant
#12
Nicholas A Zwang, Bing Ho, Yashpal S Kanwar, Brad Lewis, Matthew Cusick, John J Friedewald, Lorenzo Gallon
Atypical hemolytic uremic syndrome (aHUS) has gained increased visibility over several years as an important cause of renal failure. Unfortunately, diagnosis is often difficult because individual courses can be highly variable depending the causative genetic mutations. Here we present the case of a patient with a failed renal allograft and acute failure of a second allograft who was ultimately diagnosed with aHUS. Interestingly, he developed early de novo donor specific antibodies (DSA) after the second renal transplant in context of likely recurrent aHUS...
February 21, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28221950/multistate-outbreak-of-escherichia-coli-o157-h7-infections-associated-with-consumption-of-fresh-spinach-united-states-2006
#13
Umid M Sharapov, Arthur M Wendel, Jeffrey P Davis, William E Keene, Jeffrey Farrar, Samir Sodha, Eija Hyytia-Trees, Molly Leeper, Peter Gerner-Smidt, Patricia M Griffin, Chris Braden
During September to October, 2006, state and local health departments and the Centers for Disease Control and Prevention investigated a large, multistate outbreak of Escherichia coli O157:H7 infections. Case patients were interviewed regarding specific foods consumed and other possible exposures. E. coli O157:H7 strains isolated from human and food specimens were subtyped using pulsed-field gel electrophoresis and multiple-locus variable-number tandem repeat analyses (MLVA). Two hundred twenty-five cases (191 confirmed and 34 probable) were identified in 27 states; 116 (56%) case patients were hospitalized, 39 (19%) developed hemolytic uremic syndrome, and 5 (2%) died...
December 2016: Journal of Food Protection
https://www.readbyqxmd.com/read/28220235/successful-discontinuation-of-eculizumab-under-immunosuppressive-therapy-in-deap-hus
#14
Agnes Hackl, Rasmus Ehren, Michael Kirschfink, Peter F Zipfel, Bodo B Beck, Lutz T Weber, Sandra Habbig
BACKGROUND: Deficiency of complement factor H-related plasma proteins and complement factor H autoantibody-positive hemolytic uremic syndrome (DEAP-HUS), which is characterized by the deficiency of complement-factor H-related (CFHR) plasma proteins and the subsequent formation of autoantibodies against complement factor H (CFH), has been reported to have an adverse outcome in one third of patients. Therapy options include prompt removal of antibodies by plasma exchange and immunosuppressive therapy...
February 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28215731/complementopathies
#15
REVIEW
Andrea C Baines, Robert A Brodsky
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies"...
February 6, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28211788/hemolytic-uremic-syndrome-complicated-by-clostridium-septicum-bacteremia-and-new-onset-type-1-diabetes-mellitus-report-of-a-case%C3%A2
#16
Anna C Iddings, Asha N Shenoi, Alba Morales Pozzo, Stefan G Kiessling
We report the unusual case of a 5-year-old male hospitalized for management of diabetic ketoacidosis (DKA) and new-onset type 1 diabetes mellitus (T1DM) who developed acute renal injury secondary to hemolytic uremic syndrome (HUS). He was diagnosed with Shiga toxin-producing entero-hemorrhagic <i>Escherichia coli</i> (EHEC) 0157:H7-positive HUS 48 hours after being diagnosed with new-onset T1DM/DKA. His hospital course was complicated by <i>Clostridium septicum</i> sepsis with colonic perforation...
April 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#17
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#18
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28199087/expression-purification-and-properties-of-a-human-arachidonoyl-specific-isoform-of-diacylglycerol-kinase
#19
William Jennings, Sejal Doshi, Prasanta Kumar Hota, Aaron Prodeus, Stephanie Black, Richard M Epand
Diacylglycerol kinase ε (DGKε) catalyzes the phosphorylation of diacylglycerol, producing phosphatidic acid. DGKε demonstrates exquisite specificity for the acyl chains of diacylglycerol. This contributes to the enrichment of particular acyl chains within the lipids of the phosphatidylinositol cycle. Phosphatidylinositol is highly enriched with 1-stearoyl-2-arachidonoyl, which is important for maintaining cellular health. Dysregulation of DGKε perturbs lipid signaling and biosynthesis, which has been linked to epilepsy, Huntington's disease, and heart disease...
February 24, 2017: Biochemistry
https://www.readbyqxmd.com/read/28198690/thrombotic-microangiopathies-similar-presentations-different-therapies
#20
REVIEW
Gerald B Appel
Thrombotic thrombocytopenic purpura, Shiga toxin hemolytic uremic syndrome, atypical hemolytic uremic syndrome, and antiphospholipid syndrome are thrombotic microangiopathies that present similarly but arise from different causes. Management depends on distinguishing them promptly and providing targeted therapy.
February 2017: Cleveland Clinic Journal of Medicine
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