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https://www.readbyqxmd.com/read/28520790/correction-a-novel-quantitative-hemolytic-assay-coupled-with-restriction-fragment-length-polymorphisms-analysis-enabled-early-diagnosis-of-atypical-hemolytic-uremic-syndrome-and-identified-unique-predisposing-mutations-in-japan
#1
Yoko Yoshida, Toshiyuki Miyata, Masanori Matsumoto, Hiroko Shirotani-Ikejima, Yumiko Uchida, Yoshifumi Ohyama, Tetsuro Kokubo, Yoshihiro Fujimura
[This corrects the article DOI: 10.1371/journal.pone.0124655.].
2017: PloS One
https://www.readbyqxmd.com/read/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#2
Chakri Gavva, Prapti Patel, Yu-Min Shen, Eugene Frenkel, Ravi Sarode
Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency...
April 27, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28511109/sensitive-detection-of-viable-escherichia-coli-o157-h7-from-foods-using-a-luciferase-reporter-phage-phiv10lux
#3
Jinwoo Kim, Minsik Kim, Seongmi Kim, Sangryeol Ryu
Escherichia coli O157:H7, a major foodborne pathogen, is a major public health concern associated with life-threatening diseases such as hemolytic uremic syndrome. To alleviate this burden, a sensitive and rapid system is required to detect this pathogen in various kinds of foods. Herein, we propose a phage-based pathogen detection method to replace laborious and time-consuming conventional methods. We engineered an E. coli O157:H7-specific phage phiV10 to rapidly and sensitively detect this notorious pathogen...
May 5, 2017: International Journal of Food Microbiology
https://www.readbyqxmd.com/read/28509134/two-cases-of-atypical-hemolytic-uremic-syndrome-ahus-and-eosinophilic-granulomatosis-with-polyangiitis-egpa-a-possible-relationship
#4
Mercedes Cao, Tamara Ferreiro, Bruna N Leite, Francisco Pita, Luis Bolaños, Francisco Valdés, Angel Alonso, Eduardo Vázquez, Juan Mosquera, María Trigás, Santiago Rodríguez
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509125/a-case-of-pr3-anca-positive-anti-gbm-disease-associated-with-intrarenal-arteritis-and-thrombotic-microangiopathy
#5
Shun Manabe, Mayuko Banno, Marie Nakano, Teruhiro Fujii, Yukio Kakuta, Kosaku Nitta, Michiyasu Hatano
Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) is occasionally reported and termed "double positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA, and some of the MPO-ANCA-positive cases reveal intrarenal arteritis indicating an ANCA-associated renal lesion. In contrast, proteinase 3 (PR3)-ANCA-positive "double positive" disease had rarely been reported, and as far as we know, none of the cases showed intrarenal arteritis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508968/dialysis-disequilibrium-leading-to-posterior-reversible-encephalopathy-syndrome-in-chronic-renal-failure
#6
Pratim Sengupta, Sumanta Biswas
Dialysis disequilibrium syndrome is a neurological adverse effect of acute hemodialysis in advanced uremic patients. Dialysis disequilibrium has a wide spectrum of clinical manifestations starting from subtle uneasiness, confusion, to florid and complex life threatening neurological deficit. In this case study, we present a patient who developed sudden cortical blindness following hemodialysis due to posterior reversible encephalopathy, which is a rare presentation of dialysis disequilibrium syndrome.
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508588/at-the-cross-section-of-thrombotic-microangiopathy-and-atypical-hemolytic-uremic-syndrome-a-narrative-review-of-differential-diagnostics-and-a-problematization-of-nomenclature
#7
REVIEW
Alexander Åkesson, Eva Zetterberg, Jenny Klintman
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigger (e.g. infection) is needed for the excessive response to develop...
May 16, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#8
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28501299/severe-hypertension-with-renal-thrombotic-microangiopathy-what-happened-to-the-usual-suspect
#9
Steven Van Laecke, Wim Van Biesen
Patients with atypical hemolytic uremic syndrome (aHUS) and malignant hypertension can both present with concomitant hypertension and thrombotic microangiopathy (TMA), rendering policy decisions complex. Timmermans et al. report that patients with severe hypertension and renal TMA might have unrecognized aHUS with underlying complement abnormalities. Based on this, they assert that all patients presenting with severe hypertension and renal TMA should be evaluated for aHUS. It remains uncertain whether this holds equally true for patients with malignant hypertension and renal TMA...
June 2017: Kidney International
https://www.readbyqxmd.com/read/28485072/therapeutic-plasma-exchange-in-children-one-center-s-experience
#10
Gerard Cortina, Violeta Ojinaga, Thomas Giner, Magdalena Riedl, Siegfried Waldegger, Alejandra Rosales, Raphaela Trojer, Johannes Hofer
BACKGROUND: Therapeutic plasma exchange (TPE) has evolved to an accepted therapy for selected indications. However, it is technically challenging in children. Moreover, data on safety and efficacy are mainly derived from adult series. The aim of this study was to review the procedure in the context of clinical indications, effectiveness, and safety. STUDY DESIGN AND METHODS: All TPE procedures performed at a tertiary care hospital during a 12-year period (2005-2016) were retrospectively evaluated...
May 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28473385/draft-whole-genome-sequences-of-multidrug-resistant-escherichia-coli-o157-h7-strains-isolated-from-feedlot-cattle-treated-with-growth-promoting-agents
#11
Muhammad A Rehman, Catherine Carrillo, François Malouin, Moussa S Diarra
Enterohemorrhagic Escherichia coli serotype O157:H7 is a major cause of foodborne outbreaks and hemolytic-uremic syndrome. Here, we report the draft genome sequences of three antibiotic-resistant E. coli O157:H7 strains isolated from feedlot cattle. These draft genome sequences will aid in the development of sequence-based tools for the detection of virulence and antimicrobial resistance genotypes.
May 4, 2017: Genome Announcements
https://www.readbyqxmd.com/read/28469995/computational-analysis-of-host-pathogen-protein-interactions-between-humans-and-different-strains-of-enterohemorrhagic-escherichia-coli
#12
Tungadri Bose, K V Venkatesh, Sharmila S Mande
Serotype O157:H7, an enterohemorrhagic Escherichia coli (EHEC), is known to cause gastrointestinal and systemic illnesses ranging from diarrhea and hemorrhagic colitis to potentially fatal hemolytic uremic syndrome. Specific genetic factors like ompA, nsrR, and LEE genes are known to play roles in EHEC pathogenesis. However, these factors are not specific to EHEC and their presence in several non-pathogenic strains indicates that additional factors are involved in pathogenicity. We propose a comprehensive effort to screen for such potential genetic elements, through investigation of biomolecular interactions between E...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28461395/eculizumab-cessation-in-atypical-hemolytic-uremic-syndrome
#13
Samuel A Merrill, Zachary D Brittingham, Xuan Yuan, Alison R Moliterno, C John Sperati, Robert A Brodsky
No abstract text is available yet for this article.
May 1, 2017: Blood
https://www.readbyqxmd.com/read/28458317/long-term-eculizumab-treatment-contributes-to-recovery-from-end-stage-renal-disease-caused-by-atypical-hemolytic-uremic-syndrome
#14
Yuji Yamada, Ryohei Abe, Yutaka Okano, Yoshitaka Miyakawa
We experienced a favorable outcome in an adult case of atypical hemolytic uremic syndrome (aHUS) after long-term eculizumab treatment. A 38-year-old Japanese man with a history of central retinal vein occlusion was admitted to our hospital with progressive dyspnea. He was found to have non-immune hemolytic anemia, thrombocytopenia, and acute renal failure two weeks after an episode of the common cold. Plasma exchange was ineffective; therefore, we initiated eculizumab after we excluded other thrombotic microangiopathies...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28455885/an-extremely-rare-splice-site-mutation-in-the-gene-encoding-complement-factor-i-in-a-patient-with-atypical-hemolytic-uremic-syndrome
#15
Tina S Ipe, Jooeun Lim, Meredith Anne Reyes, Mike Ero, Christopher Leveque, Bradley Lewis, Jamey Kain
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure. The disease is difficult to diagnose due to its similarity with other hematologic disorders, such as thrombotic thrombocytopenic purpura (TTP). However, genetic mutations are found in 50-70% of patients with aHUS and can be useful in its diagnosis. STUDY DESIGN AND METHODS: A 40-year-old male presented to our hospital with acute kidney injury, evidenced by high creatinine levels (8...
April 28, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28454234/gemcitabine-associated-thrombotic-microangiopathy-in-a-patient-with-lung-cancer-a-case-report
#16
Florence Lai-Tiong, Yann Duval, Francois Krabansky
Gemcitabine is frequently used for the treatment of a number of different cancer types. Gemcitabine-related thrombotic microangiopathy (TMA) has rarely been described, but it is a life-threatening complication. The incidence of the complication varies between 0.015 and 1.4%. The present study reports the case of a 63-year-old Caucasian male who was treated with 3 cycles of carboplatin plus gemcitabine, followed by 7 cycles of gemcitabine only, and developed clinical symptoms that, together with laboratory findings, were compatible with a diagnosis of hemolytic uremic syndrome TMA...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#17
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#18
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28439485/treatment-of-atypical-hemolytic-uremic-syndrome-in-the-era-of-eculizumab
#19
Rawaa Ebrahem, Salam Kadhem, Quoc Truong
Hemolytic-uremic syndrome (HUS) is the triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury (AKI); the main cause of multi-organ failure is related to thrombotic microangiopathy (TMA). Atypical HUS (aHUS) is a disease of uncontrolled complement activation associated with a high mortality rate and most cases progress to end-stage renal disease. About 50% of patients with this syndrome carry mutations in genes that encode complement proteins. Also, aHUS constitutes an over-activation of the complement pathway which is either inherited, acquired, or both...
March 23, 2017: Curēus
https://www.readbyqxmd.com/read/28439081/long-lasting-neutralization-of-c5-by-sky59-a-novel-recycling-antibody-is-a-potential-therapy-for-complement-mediated-diseases
#20
Taku Fukuzawa, Zenjiro Sampei, Kenta Haraya, Yoshinao Ruike, Meiri Shida-Kawazoe, Yuichiro Shimizu, Siok Wan Gan, Machiko Irie, Yoshinori Tsuboi, Hitoshi Tai, Tetsushi Sakiyama, Akihisa Sakamoto, Shinya Ishii, Atsuhiko Maeda, Yuki Iwayanagi, Norihito Shibahara, Mitsuko Shibuya, Genki Nakamura, Takeru Nambu, Akira Hayasaka, Futa Mimoto, Yuu Okura, Yuji Hori, Kiyoshi Habu, Manabu Wada, Takaaki Miura, Tatsuhiko Tachibana, Kiyofumi Honda, Hiroyuki Tsunoda, Takehisa Kitazawa, Yoshiki Kawabe, Tomoyuki Igawa, Kunihiro Hattori, Junichi Nezu
Dysregulation of the complement system is linked to the pathogenesis of a variety of hematological disorders. Eculizumab, an anti-complement C5 monoclonal antibody, is the current standard of care for paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS). However, because of high levels of C5 in plasma, eculizumab has to be administered biweekly by intravenous infusion. By applying recycling technology through pH-dependent binding to C5, we generated a novel humanized antibody against C5, SKY59, which has long-lasting neutralization of C5...
April 24, 2017: Scientific Reports
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