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Hemolytic uremic syndrome

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https://www.readbyqxmd.com/read/28993505/factor-h-competitor-generated-by-gene-conversion-events-associates-with-atypical-hemolytic-uremic-syndrome
#1
Elena Goicoechea de Jorge, Agustín Tortajada, Sheila Pinto García, Sara Gastoldi, Héctor Martín Merinero, Jesús García-Fernández, Emilia Arjona, Mercedes Cao, Giuseppe Remuzzi, Marina Noris, Santiago Rodríguez de Córdoba
Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy caused by complement pathogenic variants, mainly affects the kidney microvasculature. A retrospective genetic analysis in our aHUS cohort (n=513) using multiple ligation probe amplification uncovered nine unrelated patients carrying a genetic abnormality in the complement factor H related 1 gene (CFHR1) that originates by recurrent gene conversion events between the CFH and CFHR1 genes. The novel CFHR1 mutants encode an FHR-1 protein with two amino acid substitutions, L290S and A296V, converting the FHR-1 C terminus into that of factor H (FH)...
October 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28991131/recurrent-atypical-hemolytic-uremic-syndrome-in-children-with-acute-lymphoblastic-leukemia-undergoing-maintenance-chemotherapy
#2
Geoffrey Cheng, Bulent Ozgonenel, Kanta Bhambhani, Gaurav Kapur, Richard J Smith, Süreyya Savaşan
Chemotherapy-associated myelosuppression and renal dysfunction is not uncommon during childhood acute lymphoblastic leukemia (ALL) therapy. Here we report 2 cases of atypical hemolytic uremic syndrome (aHUS) presenting with pancytopenia and renal dysfunction that developed during maintenance chemotherapy characterized by hypocomplementemia. Both cases experienced recurrence after resolution of the initial aHUS episode upon resumption of chemotherapy, raising a possible contributory role for chemotherapy in the disease pathogenesis...
October 4, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28983018/phase-1-study-of-the-anti-cd22-immunotoxin-moxetumomab-pasudotox-for-childhood-acute-lymphoblastic-leukemia
#3
MULTICENTER STUDY
Alan S Wayne, Nirali N Shah, Deepa Bhojwani, Lewis B Silverman, James A Whitlock, Maryalice Stetler-Stevenson, Weili Sun, Meina Liang, Jie Yang, Robert J Kreitman, Mark C Lanasa, Ira Pastan
Novel therapies are needed to overcome chemotherapy resistance for children with relapsed/refractory acute lymphoblastic leukemia (ALL). Moxetumomab pasudotox is a recombinant anti-CD22 immunotoxin. A multicenter phase 1 study was conducted to determine the maximum-tolerated cumulative dose (MTCD) and evaluate safety, activity, pharmacokinetics, and immunogenicity of moxetumomab pasudotox in children, adolescents, and young adults with ALL (N = 55). Moxetumomab pasudotox was administered as a 30-minute IV infusion at doses of 5 to 50 µg/kg every other day for 6 (cohorts A and B) or 10 (cohort C) doses in 21-day cycles...
October 5, 2017: Blood
https://www.readbyqxmd.com/read/28982104/repair-of-an-autologous-saphenous-vein-graft-aneurysm-ten-years-after-renal-artery-reconstruction-during-live-donor-renal-transplantation
#4
Markus Oelschlaeger, Ioannis Sokolakis, Charis Kalogirou, Lea Frey, Hubertus Riedmiller, Hubert Kübler, Richard Kellersmann, Daniel Vergho
Saphenous vein graft (SVG) aneurysms (SVGA) after renal transplantation represents a rare vascular complication with subsequent challenging multidisciplinary treatment. We present a case of a 30-year-old female who received a live donor kidney transplantation for end-stage renal disease that was caused due to the hemolytic uremic syndrome. Postoperatively, an insufficient graft perfusion due to an arterial kinking was noted and repaired using an autologous SVG interposition. Ten years later, a 3-cm aneurysm of the SVG at the anastomotic site with the common iliac artery was discovered...
October 6, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28981517/a-recombinant-o-polysaccharide-protein-conjugate-approach-to-develop-highly-specific-monoclonal-antibodies-to-shiga-toxin-producing-escherichia-coli-o157-and-o145-serogroups
#5
Daniela S Castillo, Diego A Rey Serantes, Luciano J Melli, Andrés E Ciocchini, Juan E Ugalde, Diego J Comerci, Alejandro Cassola
Shiga toxin-producing Escherichia coli (STEC) is the major etiologic agent of hemolytic-uremic syndrome (HUS). The high rate of HUS emphasizes the urgency for the implementation of primary prevention strategies to reduce its public health impact. Argentina shows the highest rate of HUS worldwide, being E. coli O157 the predominant STEC-associated HUS serogroup (>70%), followed by E. coli O145 (>9%). To specifically detect these serogroups we aimed at developing highly specific monoclonal antibodies (mAbs) against the O-polysaccharide (O-PS) section of the lipopolysaccharide (LPS) of the dominant STEC-associated HUS serogroups in Argentina...
2017: PloS One
https://www.readbyqxmd.com/read/28975130/multiple-myeloma-as-the-underlying-cause-of-thrombotic-microangiopathy-leading-to-acute-kidney-injury-revisiting-a-very-rare-entity
#6
Savneek Chugh, Asim Kichloo, Firas Jafri, Liga Yusvirazi, Robert Lerner
Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia, leading to end-organ ischemia and infarction, affecting particularly the kidney and brain. TMA is a pathological feature of a number of clinical disorders including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and atypical hemolytic uremic syndrome. Rare but important, TMA may also occur in malignancy, connective tissue disease, malignant hypertension, and renal transplantation (rejection or drug toxicity)...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28970221/characterization-of-sorbitol-fermenting-enterohemorrhagic-escherichia-coli-o157-h-from-czech-patients-identifies-novel-plasmid-composition-not-previously-seen-in-german-isolates
#7
Andreas Bauwens, Monika Marejková, Barbara Middendorf-Bauchart, Rita Prager, Annelene Kossow, Wenlan Zhang, Helge Karch, Alexander Mellmann, Martina Bielaszewska
Sorbitol-fermenting (SF) enterohemorrhagic Escherichia coli (EHEC) O157:H(-) strains, first identified in Germany, have emerged as important pathogens throughout Europe. Besides chromosomally encoded Shiga toxin 2a, the major virulence factor, several putative virulence loci including the hly, etp, and sfp operons encoding EHEC hemolysin, type II secretion system, and Sfp fimbriae, respectively, are located on a 121 kb plasmid pSFO157 in German strains. Here, we report novel SF EHEC O157:H(-) strains isolated from patients in the Czech Republic...
September 29, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28968866/hemolytic-uremic-syndrome-associated-with-pneumococci-in-children-an-elusive-mystery-now-explained
#8
Kristian Riesbeck
No abstract text is available yet for this article.
August 26, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28968817/streptococcus-pneumoniae-from-patients-with-hemolytic-uremic-syndrome-binds-human-plasminogen-via-the-surface-protein-pspc-and-uses-plasmin-to-damage-human-endothelial-cells
#9
Christian Meinel, Giuseppina Spartà, Hans-Martin Dahse, Franziska Hörhold, Rainer König, Martin Westermann, Sina M Coldewey, Zoltán Cseresnyés, Marc Thilo Figge, Sven Hammerschmidt, Christine Skerka, Peter F Zipfel
Pneumococcal hemolytic uremic syndrome (HUS) in children is caused by infections with Streptococcus pneumoniae. Because endothelial cell damage is a hallmark of HUS, we studied how HUS-inducing pneumococci derived from infant HUS patients during the acute phase disrupt the endothelial layer. HUS pneumococci efficiently bound human plasminogen. These clinical isolates of HUS pneumococci efficiently bound human plasminogen via the bacterial surface proteins Tuf and PspC. When activated to plasmin at the bacterial surface, the active protease degraded fibrinogen and cleaved C3b...
August 26, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28959735/zebrafish-danio-rerio-as-a-vertebrate-model-host-to-study-colonization-pathogenesis-and-transmission-of-foodborne-escherichia-coli-o157
#10
Daniel H Stones, Alexander G J Fehr, Laurel Thompson, Jacqueline Rocha, Nicolas Perez-Soto, Vipin T P Madhavan, Kerstin Voelz, Anne Marie Krachler
Foodborne infections with enterohemorrhagic Escherichia coli (EHEC) are a major cause of diarrheal illness in humans and can lead to severe complications such as hemolytic uremic syndrome. Cattle and other ruminants are the main reservoir of EHEC, which enters the food chain through contaminated meat, dairy, or vegetables. Here, we describe the establishment of a vertebrate model for foodborne EHEC infection, using larval zebrafish (Danio rerio) as a host and the protozoan prey Paramecium caudatum as a vehicle...
September 2017: MSphere
https://www.readbyqxmd.com/read/28947259/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-treatment-and-prognosis
#11
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Major achievements in the understanding of thrombotic microangiopathies (TMA) have not only resulted in a reclassification of TMA but most of all they have culminated in the design of new treatments and have enabled clinicians to better delineate their prognosis. Recent multicenter studies have improved our understanding of the prognosis of atypical hemolytic and uremic syndromes (aHUS). More specifically, they have highlighted the role of genetic testing on predicting the recurrence of aHUS, the risk of chronic kidney disease and the recurrence following kidney transplantation...
September 22, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28943538/hemolytic-uremic-syndrome-associated-encephalopathy-successfully-treated-with-corticosteroids
#12
Takashi Hosaka, Kiyotaka Nakamagoe, Akira Tamaoka
The encephalopathy that occurs in association with hemolytic uremic syndrome (HUS), which is caused by enterohemorrhagic Escherichia coli (E. coli), has a high mortality rate and patients sometimes present sequelae. We herein describe the case of a 20-year-old woman who developed encephalopathy during the convalescent stage of HUS caused by E.coli O26. Hyperintense lesions were detected in the pons, basal ganglia, and cortex on diffusion-weighted brain MRI. From the onset of HUS encephalopathy, we treated the patient with methylprednisolone (mPSL) pulse therapy alone...
September 25, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28941939/complete-functional-characterization-of-disease-associated-genetic-variants-in-the-complement-factor-h-gene
#13
Héctor Martín Merinero, Sheila Pinto García, Jesús García-Fernández, Emilia Arjona, Agustín Tortajada, Santiago Rodríguez de Córdoba
Genetic analyses in atypical hemolytic uremic syndrome (aHUS) and C3-glomerulopathy (C3G) patients have provided an excellent understanding of the genetic component of the disease and informed genotype-phenotype correlations supporting an individualized approach to patient management and treatment. In this context, a correct categorization of the disease-associated gene variants is critical to avoid detrimental consequences for patients and their relatives. Here we describe a comprehensive procedure to measure levels and functional activity of complement regulator factor H (FH) encoded by CFH, the commonest genetic factor associated with aHUS and C3G, and present the results of the analysis of 28 uncharacterized, disease-associated, FH variants...
September 20, 2017: Kidney International
https://www.readbyqxmd.com/read/28939980/targeted-exome-sequencing-in-anti-factor-h-antibody-negative-hus-reveals-multiple-variations
#14
R W Thergaonkar, Ankita Narang, Bahadur Singh Gurjar, Pradeep Tiwari, Mamta Puraswani, Himanshi Saini, Aditi Sinha, Binuja Varma, Mitali Mukerji, Pankaj Hari, Arvind Bagga
BACKGROUND: Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation. METHODS: We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic variations in a panel of 15 genes, i.e., CFH, CFHR1-5, CFI, CFB, C3, CD46, MASP2, DGKE, ADAMTS13, THBD and PLG using next-generation DNA sequencing and for copy number variation in CFHR1-3...
September 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28935743/genome-sequence-for-shiga-toxin-producing-escherichia-coli-o26-h11-associated-with-a-cluster-of-hemolytic-uremic-syndrome-cases-in-south-africa-2017
#15
Anthony M Smith, Kalule J Bosco, Mark P Nicol, Jackie Kleynhans, Mignon McCulloch, Sanelisiwe T Duze, Arshad Ismail, Mushal Allam, Nomsa P Tau, Karen H Keddy
Shiga toxin-producing Escherichia coli (STEC) strains are primarily foodborne pathogens that may cause diarrheal outbreaks and are associated with severe complications, specifically hemolytic-uremic syndrome (HUS). We report here genome sequence data for STEC O26:H11, which is associated with a cluster of cases of HUS, a rarely described syndrome in South Africa.
September 21, 2017: Genome Announcements
https://www.readbyqxmd.com/read/28932128/a-suspected-case-of-autoinduction-of-voriconazole-metabolism-in-a-patient-with-cerebral-aspergillosis
#16
Martin J Ferguson, Maria L Randles, Declan G de Freitas
OBJECTIVE: This study aims to report a case of accelerated metabolism of voriconazole in a patient with cerebral aspergillosis. CASE SUMMARY: A 36-year-old woman developed cerebral aspergillosis after immunosuppressive treatment for suspected atypical hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. She was treated with voriconazole using therapeutic drug monitoring to guide dosing. After an initial high level, her dose was reduced, but over the following weeks, she required several dose increases in order to achieve a voriconazole level within the target range...
2017: Drug, Healthcare and Patient Safety
https://www.readbyqxmd.com/read/28931327/renal-recovery-years-after-initial-diagnosis-of-hemolytic-uremic-syndrome
#17
Deeksha Borkar, Christina Schutt, Priya Verghese
No abstract text is available yet for this article.
September 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/28930030/mild-illness-during-outbreak-of-shiga-toxin-producing-escherichia-coli-o157-infections-associated-with-agricultural-show-australia
#18
Bhakti R Vasant, Russell J Stafford, Amy V Jennison, Sonya M Bennett, Robert J Bell, Christine J Doyle, Jeannette R Young, Susan A Vlack, Paul Titmus, Debra El Saadi, Kari A J Jarvinen, Patricia Coward, Janine Barrett, Megan Staples, Rikki M A Graham, Helen V Smith, Stephen B Lambert
During a large outbreak of Shiga toxin-producing Escherichia coli illness associated with an agricultural show in Australia, we used whole-genome sequencing to detect an IS1203v insertion in the Shiga toxin 2c subunit A gene of Shiga toxin-producing E. coli. Our study showed that clinical illness was mild, and hemolytic uremic syndrome was not detected.
October 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28925976/shiga-toxin-therapeutics-beyond-neutralization
#19
REVIEW
Gregory Hall, Shinichiro Kurosawa, Deborah J Stearns-Kurosawa
Ribotoxic Shiga toxins are the primary cause of hemolytic uremic syndrome (HUS) in patients infected with Shiga toxin-producing enterohemorrhagic Escherichia coli (STEC), a pathogen class responsible for epidemic outbreaks of gastrointestinal disease around the globe. HUS is a leading cause of pediatric renal failure in otherwise healthy children, resulting in a mortality rate of 10% and a chronic morbidity rate near 25%. There are currently no available therapeutics to prevent or treat HUS in STEC patients despite decades of work elucidating the mechanisms of Shiga toxicity in sensitive cells...
September 19, 2017: Toxins
https://www.readbyqxmd.com/read/28922657/methods-employed-in-cytofluorometric-assessment-of-eryptosis-the-suicidal-erythrocyte-death
#20
Mohamed Jemaà, Myriam Fezai, Rosi Bissinger, Florian Lang
Suicidal erythrocyte death or eryptosis contributes to or even accounts for anemia in a wide variety of clinical conditions, such as iron deficiency, dehydration, hyperphosphatemia, vitamin D excess, chronic kidney disease (CKD), hemolytic-uremic syndrome, diabetes, hepatic failure, malignancy, arteriitis, sepsis, fever, malaria, sickle-cell disease, beta-thalassemia, Hb-C and G6PD-deficiency, Wilsons disease, as well as advanced age. Moreover, eryptosis is triggered by a myriad of xenobiotics and endogenous substances including cytotoxic drugs and uremic toxins...
September 1, 2017: Cellular Physiology and Biochemistry
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