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Hemolytic uremic syndrome

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https://www.readbyqxmd.com/read/29661469/successful-7-year-eculizumab-treatment-of-plasmapheresis-resistant-recurrent-atypical-hemolytic-uremic-syndrome-due-to-complement-factor-h-hybrid-gene-a-case-report
#1
K Vondrák, T Seeman
Atypical hemolytic-uremic syndrome (aHUS) is an extremely rare disease, and up to 70% of the patients have a genetic mutation in the encoding components of complement activation or anti-complement factor H autoantibodies. The risk of recurrence after kidney transplantation is 10% to 80%. Eculizumab, a monoclonal antibody that binds complement protein C5, has shown to be highly effective in patients with aHUS; however, there are only few reports on the efficacy and safety of long-term eculizumab treatment in children with recurrent aHUS...
April 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29659362/-recurrent-atypical-hemolytic-uremic-syndrome-after-renal-transplantation-treatment-with-eculizumab
#2
Ana B Latzke, Pehuén Fernández, Carlos Chiurchiu, Daniela Sarmantano, Javier De Arteaga, Walter Douthat, Jorge De la Fuente
Atypical hemolytic uremic syndrome (aHUS) is a rare entity. It is characterized by a thrombotic microangiopathy (nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure), with a typical histopathology of thickening of capillary and arteriolar walls and an obstructive thrombosis of the vascular lumen. The syndrome is produced by a genetic or acquired deregulation of the alternative pathway of the complement system, with high rates of end stage renal disease, post-transplant recurrence, and high mortality...
2018: Medicina
https://www.readbyqxmd.com/read/29659360/-development-of-a-product-anti-shiga-toxin-for-prevention-of-the-hemolytic-uremic-syndrome
#3
Yanina Hiriart, Romina Pardo, Lucas Bukata, Constanza Lauché, Luciana Muñoz, Mariana Colonna, Fernando Goldbaum, Santiago Sanguineti, Vanesa Zylberman
The typical hemolytic uremic syndrome (HUS) is an orphan disease caused by Shiga toxin(Stx) producing Escherichia coli strains and characterized by acute kidney damage, microangiopathic hemolytic anemia and low platelet count. It is endemic in Argentina, the country with the highest incidence of HUS in the world. Stx is essential for its development and therefore, HUS is considered a toxemic non-bacteremic disorder, which could be treated with antibodies. Herein we describe the development of a new treatment capable of neutralizing the toxic effect of Stx and its variants...
2018: Medicina
https://www.readbyqxmd.com/read/29657197/adamts-13-level-in-children-with-severe-diarrhea-associated-hemolytic-uremic-syndrome-unmasking-new-association
#4
Naglaa A Khalifa, Heba H Gawish, Noora A Khalifa, Doaa M Tawfeek, Saed M Morsy
Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657196/atypical-hemolytic-uremic-syndrome-a-monocentric-adult-tunisian-study-and-review-of-literature
#5
R Kherder-Elfekih, M Hajji, L Ben Fatma, H Jebali, I Mami, W Smaoui, M Krid, L Rais, S Beji, K Zouaghi, M Sellami-Kallel
Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657193/atypical-hemolytic-uremic-syndrome-laboratory-characteristics-complement-amplifying-conditions-renal-biopsy-and-genetic-mutations
#6
REVIEW
Mohammad A Hossain, Anmol Cheema, Sheila Kalathil, Ravneet Bajwa, Eric J Costanzo, James Cosentino, Jennifer Cheng, Tushar Vachharajani, Arif Asif
Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29649283/design-and-preclinical-characterization-of-alxn1210-a-novel-anti-c5-antibody-with-extended-duration-of-action
#7
Douglas Sheridan, Zhao-Xue Yu, Yuchun Zhang, Rekha Patel, Fang Sun, Melissa A Lasaro, Keith Bouchard, Bruce Andrien, Andre Marozsan, Yi Wang, Paul Tamburini
Eculizumab, a monoclonal antibody (mAb) directed against complement protein C5, is considered to be the current standard of care for patients with paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome. This study describes the generation and preclinical attributes of ALXN1210, a new long-acting anti-C5 mAb, obtained through select modifications to eculizumab to both largely abolish target-mediated drug disposition (TMDD) and increase recycling efficiency via the neonatal Fc receptor (FcRn)...
2018: PloS One
https://www.readbyqxmd.com/read/29644059/mutations-in-membrane-cofactor-protein-cd46-gene-in-indian-children-with-hemolytic-uremic-syndrome
#8
Priyanka Khandelwal, Shweta Birla, Divya Bhatia, Mamta Puraswani, Himanshi Saini, Aditi Sinha, Pankaj Hari, Arundhati Sharma, Arvind Bagga
Background: Mutations in the CD46 gene account for an important proportion of patients with atypical hemolytic uremic syndrome (aHUS) who characteristically show multiple relapses, no response to plasma exchange and low recurrence risk in allograft. We screened for mutations in CD46 in patients with and without circulating anti-factor H (FH) antibodies-associated aHUS. Methods: We estimated CD46 surface expression by flow cytometry and sequenced the CD46 gene in 23 and 56 patients with and without circulating anti-FH antibodies, respectively...
April 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29617871/clinical-and-laboratory-predictors-of-shiga-toxin-producing-escherichia-coli-infection-in-children-with-bloody-diarrhea
#9
Ryan S McKee, Phillip I Tarr, Dennis J Dietzen, Rachit Chawla, David Schnadower
Objectives: Children with acute bloody diarrhea are at risk of being infected with Shiga toxin-producing Escherichia coli (STEC) and of progression to hemolytic uremic syndrome. Our objective was to identify clinical and laboratory factors associated with STEC infection in children who present with acute bloody diarrhea. Methods: We performed a prospective cohort study of consecutive children younger than 18 years who presented with acute (<2-week duration) bloody diarrhea between August 1, 2013, and August 1, 2014...
April 2, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29615989/intestinal-pathogenic-escherichia-coli-insights-for-vaccine-development
#10
REVIEW
Maricarmen Rojas-Lopez, Ricardo Monterio, Mariagrazia Pizza, Mickaël Desvaux, Roberto Rosini
Diarrheal diseases are one of the major causes of mortality among children under five years old and intestinal pathogenic Escherichia coli (InPEC) plays a role as one of the large causative groups of these infections worldwide. InPECs contribute significantly to the burden of intestinal diseases, which are a critical issue in low- and middle-income countries (Asia, Africa and Latin America). Intestinal pathotypes such as enteropathogenic E. coli (EPEC) and enterotoxigenic E. coli (ETEC) are mainly endemic in developing countries, while ETEC strains are the major cause of diarrhea in travelers to these countries...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29610995/extra-renal-manifestations-of-atypical-hemolytic-uremic-syndrome-in-children
#11
Kibriya Fidan, Nilüfer Göknar, Bora Gülhan, Engin Melek, Zeynep Y Yıldırım, Esra Baskın, Mutlu Hayran, Kaan Gülleroglu, Zeynep B Özçakar, Fatih Ozaltin, Oguz Soylemezoglu
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry...
April 2, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29606600/growth-advantage-of-escherichia-coli-o104-h4-strains-on-5-n-acetyl-9-o-acetyl-neuraminic-acid-as-a-carbon-source-is-dependent-on-heterogeneous-phage-borne-nans-p-esterases
#12
Nadja Saile, Lisa Schwarz, Kristina Eißenberger, Jochen Klumpp, Florian W Fricke, Herbert Schmidt
Enterohemorrhagic E. coli (EHEC) are serious bacterial pathogens which are able to cause a hemorrhagic colitis or the life-threatening hemolytic-uremic syndrome (HUS) in humans. EHEC strains can carry different numbers of phage-borne nanS-p alleles that are responsible for acetic acid release from mucin from bovine submaxillary gland and 5-N-acetyl-9-O-acetyl neuraminic acid (Neu5,9Ac2 ), a carbohydrate present in mucin. Thus, Neu5,9Ac2 can be transformed to 5-N-acetyl neuraminic acid, an energy source used by E...
March 26, 2018: International Journal of Medical Microbiology: IJMM
https://www.readbyqxmd.com/read/29594148/c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-two-important-manifestations-of-complement-system-dysfunction
#13
Ravneet Bajwa, John A DePalma, Taimoor Khan, Anmol Cheema, Sheila A Kalathil, Mohammad A Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif
The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594146/early-conversion-from-tacrolimus-to-belatacept-in-a-highly-sensitized-renal-allograft-recipient-with-calcineurin-inhibitor-induced-de-novo-post-transplant-hemolytic-uremic-syndrome
#14
Vasishta S Tatapudi, Bonnie E Lonze, Ming Wu, Robert A Montgomery
Background: Kidney transplantation is the first-line therapy for patients with end-stage renal disease since it offers greater long-term survival and improved quality of life when compared to dialysis. The advent of calcineurin inhibitor (CNI)-based maintenance immunosuppression has led to a clinically significant decline in the rate of acute rejection and better short-term graft survival rates. However, these gains have not translated into improvement in long-term graft survival. CNI-related nephrotoxicity and metabolic side effects are thought to be partly responsible for this...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29582962/-atypical-hemolitic-uremic-syndrome-after-administration-of-docetaxel
#15
Alessandro Francesco Colombo, Rosalia Boito, Francesca Bova, Sebastiano Greco, Rosita Lucà, Arcangelo Sellaro, Giuseppe Rizzuto
The purpose of this study is to describe the clinical case of a patient suffering from a gland carcinoma with bilateral inguinal and pelvic lymph node metastases in treatment with weekly administrations of Docetaxel. After two therapy cycles, the patient developed an atypical uremic hemolytic Syndrome (SEUa), treated with infusions of frozen fresh plasma, hemodialysis, and antibiotics. Because of a severe septic secondary complication on an extensive lymph node abscess, the administration of Eculizumab was not possible...
March 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29579855/renal-transplant-immunosuppression-in-patients-with-hemolytic-uremic-syndrome-four-case-reports
#16
P Galindo, M Ramirez, A Pérez Marfil, M J Espigares, J M Osoria, R Leiva, M C Ruiz Fuentes, C De Gracia, A Osuna
A high rate of recurrence has been described in atypical hemolytic uremic syndrome renal transplant recipients, favored by certain immunosuppressant drugs that can induce complement activation. We present four case series in which three patients were diagnosed pretransplantation and a fourth who had onset in the very early post-transplantation period. The patients received different immunosuppression schedules, and all had improvement after more than 2-years. We suggest the need to stratify the risk of atypical hemolytic uremic syndrome recurrence using genetic studies and the available drugs as the main factors that allow graft survival improvement today...
March 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29578925/successful-management-of-a-rare-cause-of-hemolytic-uremic-syndrome-with-eculizumab-in-a-child
#17
Caner Alparslan, Önder Yavaşcan, Belde Kasap Demir, Bahriye Atmiş, Aysun Karabay Bayazit, Göksel Leblebisatan, Elif P Öncel, Demet Alaygut, Fatma Mutlubaş, Nejat Aksu
BACKGROUND: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult. CASE: We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed...
March 23, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29572749/eculizumab-treatment-in-severe-pediatric-stec-hus-a-multicenter-retrospective-study
#18
Lucas Percheron, Raluca Gramada, Stéphanie Tellier, Remi Salomon, Jérôme Harambat, Brigitte Llanas, Marc Fila, Emma Allain-Launay, Anne-Laure Lapeyraque, Valerie Leroy, Anne-Laure Adra, Etienne Bérard, Guylhène Bourdat-Michel, Hassid Chehade, Philippe Eckart, Elodie Merieau, Christine Piètrement, Anne-Laure Sellier-Leclerc, Véronique Frémeaux-Bacchi, Chloe Dimeglio, Arnaud Garnier
BACKGROUND: Hemolytic uremic syndrome related to Shiga-toxin-secreting Escherichia coli infection (STEC-HUS) remains a common cause of acute kidney injury in young children. No specific treatment has been validated for this severe disease. Recently, experimental studies highlight the potential role of complement in STEC-HUS pathophysiology. Eculizumab (EC), a monoclonal antibody against terminal complement complex, has been used in severe STEC-HUS patients, mostly during the 2011 German outbreak, with conflicting results...
March 23, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29563942/atypical-hemolytic-uremic-syndrome-and-eculizumab-therapy-in-children
#19
REVIEW
Seong Heon Kim, Hye Young Kim, Su Young Kim
Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli -associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy...
February 2018: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/29563339/germline-mutations-in-the-alternative-pathway-of-complement-predispose-to-hellp-syndrome
#20
Arthur J Vaught, Evan M Braunstein, Jagar Jasem, Xuan Yuan, Igor Makhlin, Solange Eloundou, Andrea C Baines, Samuel A Merrill, Shruti Chaturvedi, Karin Blakemore, C John Sperati, Robert A Brodsky
BACKGROUND: HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a severe variant of hypertensive disorders of pregnancy affecting approximately 1% of all pregnancies, and has significant maternal and fetal morbidity. Previously, we showed that upregulation of the alternative pathway of complement (APC) plays a role in HELLP syndrome. We hypothesize that HELLP syndrome follows a 2-hit disease model similar to atypical hemolytic uremic syndrome (aHUS), requiring both genetic susceptibility and an environmental risk factor...
March 22, 2018: JCI Insight
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