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uremic hemolytic syndrome

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https://www.readbyqxmd.com/read/29778196/vaccination-with-killed-whole-cells-of-escherichia-coli-o157-h7-hha-mutant-emulsified-with-an-adjuvant-induced-vaccine-strain-specific-serum-antibodies-and-reduced-e-coli-o157-h7-fecal-shedding-in-cattle
#1
Vijay K Sharma, Robert G Schaut, Crystal L Loving
Escherichia coli O157:H7 (O157) can cause from a mild diarrheal illness to hemorrhagic colitis and hemolytic uremic syndrome in humans. Cattle are the primary reservoir for O157 and fecal shedding of O157 by these animals is a major risk factor in contamination of cattle hides and carcasses at slaughter. Vaccination is an important strategy to reduce fecal shedding of O157 in cattle. In this study, we evaluated the immunogenicity and efficacy of an inactivated vaccine strain of O157 formulated with an adjuvant...
June 2018: Veterinary Microbiology
https://www.readbyqxmd.com/read/29774464/eculizumab-in-stec-hus-need-for-a-proper-randomized-controlled-trial
#2
Sebastian Loos, Jun Oh, Markus J Kemper
Hemolytic uremic syndrome caused by Shiga toxin-producing E. coli (STEC-HUS) is often associated with a severe morbidity including neurological involvement and a mortality of 1-5%. Although STEC-HUS is often self-limited, improvement of treatment strategies is needed for cases with complications and, among others, plasma exchange/plasmapheresis and use of antibiotics have been advocated. With the availability of the complement blocker eculizumab, now a standard treatment of atypical HUS, several series have addressed its use in STEC-HUS, with variable response; randomized controlled trials are lacking...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29770083/an-outbreak-of-shiga-toxin-producing-escherichia-coli-o121-infections-associated-with-flour-canada-2016-2017-%C3%A2
#3
V Morton, J M Cheng, D Sharma, A Kearney
As of May 23, 2017, 29 cases of a new Escherichia coli O121 infection have been identified in six provinces (Alberta, British Columbia, Newfoundland and Labrador, Ontario, Quebec and Saskatchewan) linked with the consumption of uncooked flour. One additional case was identified in a U.S. resident who traveled to Canada during the exposure period. Patients' ages ranged from 2-79 years (median = 23.5 years) and 50% were female. Eight patients were hospitalized, and one developed hemolytic uremic syndrome. Because of the recent emergence of E...
July 6, 2017: Canada Communicable Disease Report, Relevé des Maladies Transmissibles Au Canada
https://www.readbyqxmd.com/read/29768958/bevacizumab-induced-atypical-hemolytic-uremic-syndrome-and-treatment-with-eculizumab
#4
Anusha Vakiti, Daulath Singh, Ravi Pilla, Muhamad A Moustafa, Kelly W Fitzpatrick
Bevacizumab (Avastin) is a recombinant humanized monoclonal antibody used for the management of various solid malignancies including colorectal, lung, brain, renal, and ovarian cancers as well as age-related macular degeneration of the eye. It is a vascular endothelial growth factor inhibitor which exhibits its action by blocking the growth of blood vessels in cancerous tissue. Common side effects include hypertension, fatigue, headaches, and increased risk of infections. Atypical hemolytic uremic syndrome is a serious side effect associated with bevacizumab due to its anti-angiogenic effect...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29765909/shiga-toxin-producing-escherichia-coli-infection-in-j%C3%A3-nk%C3%A3-ping-county-sweden-occurrence-and-molecular-characteristics-in-correlation-with-clinical-symptoms-and-duration-of-stx-shedding
#5
Xiangning Bai, Sara Mernelius, Cecilia Jernberg, Ing-Marie Einemo, Stefan Monecke, Ralf Ehricht, Sture Löfgren, Andreas Matussek
Shiga toxin-producing Escherichia coli (STEC) cause bloody diarrhea (BD), hemorrhagic colitis (HC), and even hemolytic uremic syndrome (HUS). In Nordic countries, STEC are widely spread and usually associated with gastrointestinal symptoms and HUS. The objective of this study was to investigate the occurrence of STEC in Swedish patients over 10 years of age from 2003 through 2015, and to analyze the correlation of critical STEC virulence factors with clinical symptoms and duration of stx shedding. Diarrheal stool samples were screened for presence of stx by real-time PCR...
2018: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/29753419/severe-renal-failure-and-thrombotic-microangiopathy-induced-by-malignant-hypertension-successfully-treated-with-spironolactone
#6
H M Villafuerte Ledesma, J M Peña Porta, P Iñigo Gil, P Martin Azara, J E Ruiz Lalglesia, A Tomás LaTorre, A Martínez Burillo, P Vernet Perna, R Álvarez Lipe
Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0...
May 9, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29750742/an-atypical-case-of-atypical-hemolytic-uremic-syndrome
#7
Tine Francois, Johan Vande Walle, Evelyn Dhont, Werner Keenswijk
We present the case of a 2-month-old infant presenting with pallor and laboratory results showing: hemoglobin 5.1 (10 to 1.5) g/dL, MCV 94.7 (75 to 105) fL, leukocytes 17.4 (7 to 15) ×10/μL, platelets 259 (150 to 450) ×10/μL, hyperbilirubinemia and renal dysfunction. A hemolytic anemia with tubular injury secondary to hemoglobinuria was suspected. Hyperhydration and packed cells were given but she deteriorated. Fluid overload with anuria further complicated the course necessating hemodialysis. Atypical hemolytic uremic syndrome was suspected and eculizumab was administered resulting in rapid improvement...
May 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29740447/high-complement-factor-h-related-fhr-3-levels-are-associated-with-the-atypical-hemolytic-uremic-syndrome-risk-allele-cfhr3-b
#8
Richard B Pouw, Irene Gómez Delgado, Alberto López Lera, Santiago Rodríguez de Córdoba, Diana Wouters, Taco W Kuijpers, Pilar Sánchez-Corral
Dysregulation of the complement alternative pathway (AP) is a major pathogenic mechanism in atypical hemolytic-uremic syndrome (aHUS). Genetic or acquired defects in factor H (FH), the main AP regulator, are major aHUS drivers that associate with a poor prognosis. FH activity has been suggested to be downregulated by homologous FH-related (FHR) proteins, including FHR-3 and FHR-1. Hence, their relative levels in plasma could be disease-relevant. The genes coding for FH, FHR-3, and FHR-1 ( CFH, CFHR3 , and CFHR1 , respectively) are polymorphic and located adjacent to each other on human chromosome 1q31...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29738043/complement-activation-in-atypical-hemolytic-uremic-syndrome-and-scleroderma-renal-crisis-a-critical-analysis-of-pathophysiology
#9
Roman Zuckerman, Arif Asif, Eric J Costanzo, Tushar Vachharajani
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the role of complement pathways in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis. The abundant similarities in their presentation as well as the clinical course are raising the possibility of a common underlying pathogenesis. Recent reports are emphasizing that complement pathways appear to be the unifying link...
May 7, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29735067/survival-and-interaction-of-escherichia-coli-o104-h4-on-arabidopsis-thaliana-and-lettuce-lactuca-sativa-in-comparison-to-e-coli-o157-h7-influence-of-plant-defense-response-and-bacterial-capsular-polysaccharide
#10
Hyein Jang, Karl R Matthews
Shiga toxin-producing Escherichia coli (STEC) has been associated with illnesses and outbreaks linked to fresh vegetables, prompting a growing public health concern. Most studies regarding interactions of STEC on fresh produce focused on E. coli O157:H7. Limited information is available about survival or fitness of E. coli O104:H4, non-O157 pathogen that was linked to one of the largest outbreaks of hemolytic uremic syndrome in 2011. In this study, survival of E. coli O104:H4 was evaluated on Arabidopsis thaliana plant and lettuce for 5 days compared with E...
June 2018: Food Research International
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#11
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29725629/acute-kidney-injury-in-pregnancy-the-changing-landscape-for-the-21st-century
#12
REVIEW
Swati Rao, Belinda Jim
Pregnancy-related acute kidney injury (Pr-AKI) remains a large public health problem, with decreasing incidences in developing countries but seemingly increasing incidences in the United States and Canada. These epidemiologic changes are reflective of the advances in medical and obstetric care, as well as changes in underlying maternal risk factors. The risk factors associated with advanced maternal age, such as hypertension, diabetes, chronic kidney disease, and those associated with reproductive technologies such as multiple gestations, are increasing...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29706017/distribution-of-exogenous-complement-factor-h-in-mice-in-vivo
#13
Aino R Koskinen, Zhu-Zhu Cheng, Matthew C Pickering, Kalevi Kairemo, Taru Meri, H Terence Cook, Seppo Meri, T Sakari Jokiranta
Factor H is an important regulator of complement activation in plasma and on cell surfaces in both humans and mice. If FH function is compromised, inappropriate complement activation on self-surfaces can have disastrous effects as seen in the kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Since FH constructs have been proposed to be used in treatment for these diseases, we studied the distribution of exogenous FH fragments in mice. Full-length mFH, mFH1-5 and mFH18-20 fragments were radiolabeled and their distribution was examined in WT, FH-/- and FH-/- C3-/- mice in vivo...
April 29, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29703465/thrombotic-microangiopathy-in-cancer
#14
REVIEW
Ilene Ceil Weitz
Thrombotic microangiopathy (TMA) is clinical syndrome based on the presence of thrombocytopenia (platelet count <150 K or a reduction of the platelet count by >30% from baseline) accompanied by fragmentation hemolysis (MAHA) and evidence of organ damage. It can be seen in a variety of disorders including thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), shigatoxin related hemolytic uremic syndrome (STEC-HUS). Cancer itself has long been associated with both macro and microvascular thrombosis...
April 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29702577/genotypic-features-of-clinical-and-bovine-escherichia-coli-o157-strains-isolated-in-countries-with-different-associated-disease-incidences
#15
REVIEW
Luis Pianciola, Marta Rivas
There is great geographical variation in the frequency of Escherichia coli O157 infections that correlates with important differences in the bovine reservoir of each country. Our group carried out a broad molecular characterization of human and bovine E. coli O157 strains circulating in Argentina using different methodologies. Our data allows us to conclude that in Argentina, a high homogeneity is observed in both cattle and human strains, with almost exclusive circulation of strains belonging to the hypervirulent clade 8 described by Manning...
April 27, 2018: Microorganisms
https://www.readbyqxmd.com/read/29702545/steroid-responsive-atypical-hemolytic-uremic-syndrome-triggered-by-influenza-b-infection
#16
Nupur Mittal, Robert Hartemayer, Sara Jandeska, Lisa Giordano
Atypical hemolytic uremic syndrome (aHUS) is characterized by uncontrolled complement activation leading to thrombotic microangiopathy and severe end-organ damage. The most common trigger for an episode of aHUS in the background of genetic deregulation of the alternative complement pathway is systemic infection. There are only 4 reported cases of aHUS triggered by influenza B thus far. Current accepted therapies for aHUS include plasma exchange and eculizumab. We describe a unique patient with aHUS with a rare membrane cofactor protein mutation triggered by influenza B infection, who achieved complete remission with treatment with high-dose corticosteroids after failure of plasmapheresis...
April 26, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29699703/where-are-we-with-haemolytic-uremic-syndrome
#17
REVIEW
Teresa Cavero, Marina Alonso
Haemolytic uremic syndrome (HUS) is characterised by microangiopathic haemolytic anaemia with acute kidney injury. It is currently classified into two main categories: Shiga-toxin producing E. coli-hemolytic uremic syndrome (STEC-HUS) and atypical haemolytic uremic syndrome (aHUS). Endothelial cell damage is the common pathway in HUS to developing thrombotic microangiopathy. Atypical HUS includes primary, secondary and aHUS due to metabolic diseases. In the majority of aHUS cases, hyperactivity of the alternative complement pathway plays a central role...
April 23, 2018: Medicina Clínica
https://www.readbyqxmd.com/read/29698757/overview-of-the-role-of-shiga-toxins-in-porcine-edema-disease-pathogenesis
#18
REVIEW
Natalia A Casanova, Leandro M Redondo, Gabriela C Dailoff, David Arenas, Mariano E Fernández Miyakawa
Shiga toxin-producing Escherichia coli (STEC) have been implicated as the cause of enterotoxemias, such as hemolytic uremic syndrome in humans and edema disease (ED) of pigs. Stx1 and Stx2 are the most common types found in association with illness, but only Stx2e is associated with disease in the animal host. Porcine edema disease is a serious affection which can lead to dead causing great losses of weaned piglets. Stx2e is the most frequent Stx variant found in porcine feces and is considered the key virulence factor involved in the pathogenesis of porcine edema disease...
April 23, 2018: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/29695177/clinical-manifestation-of-patients-with-atypical-hemolytic-uremic-syndrome-with-the-c3-p-i1157t-variation-in-the-kinki-region-of-japan
#19
Takeshi Matsumoto, Hidemi Toyoda, Keishirou Amano, Masahiro Hirayama, Eiji Ishikawa, Mika Fujimoto, Masaaki Ito, Kohshi Ohishi, Naoyuki Katayama, Yoko Yoshida, Masanori Matsumoto, Naohisa Kawamura, Makoto Ikejiri, Keiki Kawakami, Toshiyuki Miyata, Hideo Wada
The gain-of-function variation p.I1157T in C3 was previously identified in 8 patients with atypical hemolytic uremic syndrome (aHUS) at Mie University Hospital. In the present study, we identified another 11 patients with aHUS with this variation, including 10 pediatric patients (onset age: 1-16 years). The variation seems to be geographically concentrated around Mie Prefecture in Japan. Fifteen of the 19 patients with aHUS experienced infection as probable triggering events. All 19 patients had renal dysfunction...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29686068/recurrent-structural-variation-clustered-sites-of-selection-and-disease-risk-for-the-complement-factor-h-cfh-gene-family
#20
Stuart Cantsilieris, Bradley J Nelson, John Huddleston, Carl Baker, Lana Harshman, Kelsi Penewit, Katherine M Munson, Melanie Sorensen, AnneMarie E Welch, Vy Dang, Felix Grassmann, Andrea J Richardson, Robyn H Guymer, Tina A Graves-Lindsay, Richard K Wilson, Bernhard H F Weber, Paul N Baird, Rando Allikmets, Evan E Eichler
Structural variation and single-nucleotide variation of the complement factor H ( CFH ) gene family underlie several complex genetic diseases, including age-related macular degeneration (AMD) and atypical hemolytic uremic syndrome (AHUS). To understand its diversity and evolution, we performed high-quality sequencing of this ∼360-kbp locus in six primate lineages, including multiple human haplotypes. Comparative sequence analyses reveal two distinct periods of gene duplication leading to the emergence of four CFH -related ( CFHR ) gene paralogs ( CFHR2 and CFHR4 ∼25-35 Mya and CFHR1 and CFHR3 ∼7-13 Mya)...
April 23, 2018: Proceedings of the National Academy of Sciences of the United States of America
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