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uremic hemolytic syndrome

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https://www.readbyqxmd.com/read/28424685/analysis-of-linear-antibody-epitopes-on-factor-h-and-cfhr1-using-sera-of-patients-with-autoimmune-atypical-hemolytic-uremic-syndrome
#1
Eszter Trojnár, Mihály Józsi, Katalin Uray, Dorottya Csuka, Ágnes Szilágyi, Danko Milosevic, Vesna D Stojanović, Brankica Spasojević, Krisztina Rusai, Thomas Müller, Klaus Arbeiter, Kata Kelen, Attila J Szabó, György S Reusz, Satu Hyvärinen, T Sakari Jokiranta, Zoltán Prohászka
INTRODUCTION: In autoimmune atypical hemolytic uremic syndrome (aHUS), the complement regulator factor H (FH) is blocked by FH autoantibodies, while 90% of the patients carry a homozygous deletion of its homolog complement FH-related protein 1 (CFHR1). The functional consequence of FH-blockade is widely established; however, the molecular basis of autoantibody binding and the role of CFHR1 deficiency in disease pathogenesis are still unknown. We performed epitope mapping of FH to provide structural insight in the autoantibody recruitment on FH and potentially CFHR1...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28421309/genetic-makeup-of-shiga-toxin-producing-escherichia-coli-in-relation-to-clinical-symptoms-and-duration-of-shedding-a-microarray-analysis-of-isolates-from-swedish-children
#2
A Matussek, C Jernberg, I-M Einemo, S Monecke, R Ehricht, I Engelmann, S Löfgren, S Mernelius
Shiga toxin (Stx)-producing Escherichia coli (STECs) cause non-bloody diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome, and are the primary cause of acute renal failure in children worldwide. This study investigated the correlation of genetic makeup of STEC strains as revealed by DNA microarray to clinical symptoms and the duration of STEC shedding. All STEC isolated (n = 96) from patients <10 years of age in Jönköping County, Sweden from 2003 to 2015 were included. Isolates were characterized by DNA microarray, including almost 280 genes...
April 19, 2017: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/28419995/hemolytic-uremic-syndrome-and-kidney-transplantation-a-case-series-and-review-of-the-literature
#3
Sabrina Milan Manani, Grazia Maria Virzì, Anna Giuliani, Anna Clementi, Alessandra Brocca, Daniela Dissegna, Francesca Martino, Emanuele Stefano Giovanni d'Amore, Claudio Ronco
BACKGROUND: Hemolytic uremic syndrome (HUS) can be triggered by Shiga toxin producing Escherichia coli (STEC) infection or it can be defined as atypical HUS (aHUS) if it is related to uncontrolled complement activation. aHUS is characterized by a high incidence of recurrence after kidney transplantation, and it can also occur de novo in transplant recipients. Eculizumab is used both to prevent and to treat aHUS following kidney transplantation. In this paper, we report our centre experience and we present 4 cases of HUS in patients who underwent kidney transplantation...
April 19, 2017: Nephron
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#4
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#5
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by shiga-toxin producing E. coli (STEC) infection, atypical HUS (aHUS), usually caused by uncontrolled complement activation, or secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical (i.e. STEC-HUS) follows a gastrointestinal infection with STEC, while aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28410496/acute-kidney-injury-in-symptomatic-primary-epstein-barr-virus-infectious-mononucleosis-systematic-review
#6
Milena Moretti, Sebastiano A G Lava, Lorenzo Zgraggen, Giacomo D Simonetti, Lisa Kottanattu, Mario G Bianchetti, Gregorio P Milani
BACKGROUND AND OBJECTIVES: Textbooks and reviews do not mention the association of symptomatic primary Epstein-Barr virus infectious mononucleosis with acute kidney injury in subjects without immunodeficiency or autoimmunity. STUDY DESIGN: Stimulated by our experience with two cases, we performed a review of the literature. RESULTS: The literature documents 38 cases (26 male and 12 female individuals ranging in age from 0.3 to 51, median 18 years) of symptomatic primary Epstein-Barr virus infectious mononucleosis complicated by acute kidney injury: 27 acute interstitial nephritides, 1 jaundice-associated nephropathy, 7 myositides and 3 hemolytic uremic syndromes...
March 21, 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28405075/acquired-thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-successfully-treated-with-eculizumab
#7
Appalanaidu Sasapu, Michele Cottler-Fox, Pooja Motwani
Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with a mortality of up to 90%, if not promptly recognized and treated. We report a 64-year-old woman with this condition who presented with left-sided weakness and seizure-like activity preceded by headache and easy bruising. She did not achieve optimal response to plasma exchange, corticosteroids, rituximab, and vincristine. We initiated treatment with eculizumab, following which she had durable remission that continued for 30 months after discontinuation of the drug...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28391332/many-drugs-for-many-targets-novel-treatments-for-complement-mediated-glomerular-disease
#8
Joshua M Thurman
There is a large body of experimental and clinical evidence that complement activation contributes to glomerular injury in multiple different diseases. However, the underlying mechanisms that trigger complement activation vary from disease to disease. Immune complexes activate the classical pathway of complement in many types of glomerulonephritis, whereas the alternative pathway and mannose-binding lectin pathways are directly activated in some diseases. Eculizumab is an inhibitory antibody to C5 that has been approved for the treatment of atypical hemolytic uremic syndrome, and case reports suggest that it is also effective in other types of glomerulonephritis...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28389899/degradation-and-inactivation-of-shiga-toxins-by-nitrogen-gas-plasma
#9
Akikazu Sakudo, Yuichiro Imanishi
Shiga toxin (Stx)-producing Escherichia coli (STEC) leads to food poisoning by causing hemorrhagic colitis and hemolytic uremic syndrome. Some STEC produce Shiga toxin 1 (Stx1) and/or Shiga toxin 2 (Stx2), a relatively stable protein toxin, necessitating the development of an efficient inactivation method. Here we applied a nitrogen gas plasma apparatus to the inactivation of Stx. Samples of Stx1 and Stx2 were treated with a nitrogen gas plasma generated by a plasma device using a short high-voltage pulse applied by a static induction thyristor power supply at 1...
December 2017: AMB Express
https://www.readbyqxmd.com/read/28387984/practical-issues-in-using-eculizumab-for-children-with-atypical-hemolytic-uremic-syndrome-in-the-acute-phase-a-review-of-4-patients
#10
Chikako Terano, Kenji Ishikura, Riku Hamada, Yasuhiro Yoshida, Wataru Kubota, Yusuke Okuda, Shunsuke Shinozuka, Ryoko Harada, Sunao Iyoda, Yoshihiro Fujimura, Yuko Hamasaki, Hiroshi Hataya, Masataka Honda
AIM: Recently eculizumab, a monoclonal antibody to C5, was found to improve the disease course of atypical hemolytic uremic syndrome (aHUS) and has been recommended as the first line treatment by an international consensus guideline. However, several practical issues in the use of eculizumab for the acute phase of aHUS have yet to be resolved. METHODS: Children who received eculizumab with diagnosis of aHUS between March 2010 and December 2015 at Tokyo Metropolitan Children's Medical Center were enrolled...
April 7, 2017: Nephrology
https://www.readbyqxmd.com/read/28381151/omental-arteriopathy-in-primary-atypical-hemolytic-uremic-syndrome
#11
Natalie Ellington, Matthias T F Wolf, Jennifer Kasten, Dinesh Rakheja
No abstract text is available yet for this article.
March 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28368342/strategy-for-accurate-detection-of-escherichia-coli-o157-h7-in-ground-pork-using-a-lateral-flow-immunoassay
#12
Song Cheng, Ming-Hui Chen, Gang-Gang Zhang, Zhi-Biao Yu, Dao-Feng Liu, Yong-Hua Xiong, Hua Wei, Wei-Hua Lai
Escherichia coli O157:H7 is known to cause serious diseases including hemorrhagic colitis and hemolytic uremic syndrome. A gold nanoparticle lateral flow immunoassay (Au-LFIA) was used to detect Escherichia coli O157:H7 in ground pork samples. False-positive results were detected using Au-LFIA; a Citrobacterfreundii strain was isolated from the ground pork samples and identified by using CHROmagar(TM) plates, API 20E, and 16S RNA sequencing. Since C.freundii showed cross-reactivity with E. coli O157:H7 when Au-LFIA test strips were used, a novel method combining modified enrichment with a lateral flow immunoassay for accurate and convenient detection of E...
April 2, 2017: Sensors
https://www.readbyqxmd.com/read/28366510/controlling-the-anaphylatoxin-c5a-in-diseases-requires-a-specifically-targeted-inhibition
#13
Niels C Riedemann, Maria Habel, Jana Ziereisen, Marlen Hermann, Conny Schneider, Cyrill Wehling, Michael Kirschfink, Karim Kentouche, Renfeng Guo
The terminal complement split product C5a has been described as an important mediator in inflammatory diseases. C5a is generated upon cleavage of C5 and earlier research suggests that, besides the known C5 convertases formed upon activation of the complement pathways, various enzymes could activate C5 directly. We demonstrate that eculizumab effectively blocks C5 activation when mediated by C5-convertase formation, but fails to block C5a generation resulting from direct enzymatic cleavage by trypsin and thrombin...
March 30, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28356668/targeting-renin-angiotensin-system-in-malignant-hypertension-in-atypical-hemolytic-uremic-syndrome
#14
V Raghunathan, S K Sethi, M A Dragon-Durey, M Dhaliwal, R Raina, P Jha, S B Bansal, V Kher
Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28353273/clinical-characteristics-of-children-with-hemolytic-uremic-syndrome-in-hangzhou-china
#15
Shui-Ai Zhao, Bo-Tao Ning, Jian-Hua Mao
BACKGROUND: Hemolytic uremic syndrome (HUS) is a main cause of acute renal failure in children. This study aimed to analyze the clinical characteristics of HUS. METHODS: A retrospective analysis was performed in 46 children with sporadic HUS. RESULTS: Of the 46 HUS patients, 20 (43.5%) were diarrhea-related HUS, and 26 (56.5%) were atypical HUS. Anemia, edema, oliguria, hemoglobinuria and hypertension were the most common manifestations. Thrombocytopenia, hyponatremia, hypocalcemia, hyperkalemia, metabolic acidosis, increased fibrinogen and hypocomplementemia were found in most patients...
April 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28352987/hemolytic-uremic-syndrome-caused-by-e-coli-o157-infection
#16
Philip V Bystrom, Robert J Beck, Joseph A Prahlow
No abstract text is available yet for this article.
March 28, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28352025/eculizumab-experience-in-an-adult-patient-with-atypical-hemolytic-uremic-syndrome
#17
Funda Sari, Ayça Inci, Volkan Karakus, Banu Yilmaz, Metin Sarikaya, Refik Olmaz, Erdal Kurtoglu
Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28343354/unusual-severe-case-of-hemolytic-uremic-syndrome-due-to-shiga-toxin-2d-producing-e-coli-o80-h2
#18
Kioa L Wijnsma, Anne M Schijvens, John W A Rossen, A M D Mirjam Kooistra-Smid, Michiel F Schreuder, Nicole C A J van de Kar
BACKGROUND: Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children, with the majority of cases caused by an infection with Shiga toxin-producing Escherichia coli (STEC). Whereas O157 is still the predominant STEC serotype, non-O157 serotypes are increasingly associated with STEC-HUS. However, little is known about this emerging and highly diverse group of non-O157 serotypes. With supportive therapy, STEC-HUS is often self-limiting, with occurrence of chronic sequelae in just a small proportion of patients...
March 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341889/complement-regulation-and-kidney-diseases-recent-knowledge-of-the-double-edged-roles-of-complement-activation-in-nephrology
#19
REVIEW
Masashi Mizuno, Yasuhiro Suzuki, Yasuhiko Ito
The complement activation system plays important roles to maintain homeostasis in the host and to fight foreign invaders to protect the host. Therefore, the complement system is considered a core part of innate immunity which also cross-talks to acquired immunity. In the history of nephrology, the complement system is familiar to us, because complement protein or fragment deposition, including C3, C4, C1q, and/or C4d, is routinely estimated by immunohistochemistry to diagnose renal pathologies. The relationships between pathological mechanisms and complement activation have been investigated for renal diseases such as post-infectious glomerulonephritis, lupus nephritis, and primary membranoproliferative glomerulonephritis, which are usually accompanied by hypocomplementemia...
March 24, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28340492/hemolysis-elevated-liver-enzymes-low-platelets-syndrome-superimposed-on-hemolytic-uremic-syndrome
#20
Inês Martins, Madalena Gomes Conceição, Paulo Pereira Gomes, Nuno Clode
A pregnancy complicated by typical hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is reported. At 20 weeks of gestation, a case of HUS was diagnosed, with Shiga toxin-producing Escherichia coli identified. Plasmapheresis allowed continuation of the pregnancy for 5 weeks. Superimposed preeclampsia and HELLP syndrome were diagnosed after the establishment of nephrotic range proteinuria, hypertension and recurrence of hemolysis. This is a singular case, as it demonstrates that HELLP syndrome can superimpose upon HUS, a fact that can impact future research on reproductive immunology...
April 2017: Revista Brasileira de Ginecologia e Obstetrícia
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