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haemolytic uraemic syndrome

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https://www.readbyqxmd.com/read/28899876/treatment-and-management-of-children-with-haemolytic-uraemic-syndrome
#1
REVIEW
Patrick R Walsh, Sally Johnson
Haemolytic uraemic syndrome (HUS), comprising microangiopathic haemolytic anaemia, thrombocytopaenia and acute kidney injury, remains the leading cause of paediatric intrinsic acute kidney injury, with peak incidence in children aged under 5 years. HUS most commonly occurs following infection with Shiga toxin-producing Escherichia coli (STEC-HUS). Additionally, HUS can occur as a result of inherited or acquired dysregulation of the alternative complement cascade (atypical HUS or aHUS) and in the setting of invasive pneumococcal infection...
September 12, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28855217/decreased-severity-of-shiga-toxin-producing-escherichia-coli-haemolytic-uraemic-syndrome-stec-hus-in-a-child-with-type-1-von-willebrand-disease
#2
Matthew A Geramita, Johannes Hofer, James Cooper, Michael L Moritz
Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is characterised by haemolytic anaemia, thrombocytopenia and acute kidney injury. Von Willebrand Factor (vWF) is an important mediator of normal thrombi formation and indirect evidence suggests that vWF may play an important role in Shiga toxin-induced thrombi formation. Clinical evidence supporting the role of vWF in STEC-HUS is lacking. A 10-year-old girl with type 1 von Willebrand Disease (vWD) had a mild case of STEC-HUS, with nadir haemoglobin 7...
August 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28852487/rare-genetic-variants-in-shiga-toxin-associated-haemolytic-uraemic-syndrome-genetic-analysis-prior-to-transplantation-is-essential
#3
Frances Dowen, Katrina Wood, Alison L Brown, Jennifer Palfrey, David Kavanagh, Vicky Brocklebank
We present a case of haemolytic uraemic syndrome (HUS) in a 16-year-old female with serological evidence of acute Escherichia coli O157:H7 infection. She progressed to established renal failure and received a deceased donor kidney transplant. Shiga toxin-associated HUS (STEC-HUS) does not recur following renal transplantation, but unexpectedly this patient did experience rapid and severe HUS recurrence. She responded to treatment with the terminal complement inhibitor eculizumab and subsequent genetic analysis revealed a rare variant in a complement gene...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28748411/the-role-of-von-willebrand-factor-in-thrombotic-microangiopathy
#4
REVIEW
Damien G Noone, Magdalena Riedl, Christoph Licht
Thrombotic microangiopathy (TMA) is caused by thrombus formation in the microvasculature. The disease spectrum of TMA includes, amongst others, thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS). TTP is caused by defective cleavage of von Willebrand factor (VWF), whereas aHUS is caused by overshooting complement activation and subsequent endothelial cell (EC) injury. Despite their distinct pathophysiology, the clinical manifestation of TTP and aHUS consisting of microangiopathic haemolytic anaemia and thrombocytopenia is often similar and difficult to distinguish...
July 26, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28732694/development-and-evaluation-of-latex-agglutination-tests-for-the-detection-of-human-antibodies-to-the-lipopolysaccharides-of-verocytotoxin-producing-escherichia-coli-vtec-serogroups-o157-and-non-o157
#5
Waldemar Rastawicki, Anna Chróst, Kornelia Gielarowiec
Latex agglutination tests (LAT) were developed and evaluated for the rapid detection of LPS antibodies against E. coli serogroup O157, O26, O104, O111 and O145. The latex tests have been proved to be sensitive, fast, easy-to-perform and cost-efficient tools for the screening serodiagnosis of VTEC infections causing haemolytic-uraemic syndrome.
July 18, 2017: Journal of Microbiological Methods
https://www.readbyqxmd.com/read/28699143/glutaric-aciduria-type-1-and-acute-renal-failure-case-report-and-suggested-pathomechanisms
#6
Marcel du Moulin, Bastian Thies, Martin Blohm, Jun Oh, Markus J Kemper, René Santer, Chris Mühlhausen
Glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial matrix enzyme glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in tissues and body fluids. During catabolic crises, GA1 patients are prone to the development of striatal necrosis and a subsequent irreversible movement disorder during a time window of vulnerability in early infancy. Thus, GA1 had been considered a pure "cerebral organic aciduria" in the past. Single case reports have indicated the occurrence of acute renal dysfunction in children affected by GA1...
July 12, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28638625/early-eculizumab-use-in-atypical-haemolytic-uraemic-syndrome-in-a-jehovah-s-witness-refusing-blood-products
#7
May Al-Shaghana, Andrew Bentall, Mark D Jesky, William Lester, Graham Lipkin
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28637873/disease-linked-mutations-in-factor-h-reveal-pivotal-role-of-cofactor-activity-in-self-surface-selective-regulation-of-complement-activation
#8
COMPARATIVE STUDY
Heather Kerr, Edwin Wong, Elisavet Makou, Yi Yang, Kevin Marchbank, David Kavanagh, Anna Richards, Andrew P Herbert, Paul N Barlow
Spontaneous activation enables the complement system to respond very rapidly to diverse threats. This activation is efficiently suppressed by complement factor H (CFH) on self-surfaces but not on foreign surfaces. The surface selectivity of CFH, a soluble protein containing 20 complement-control protein modules (CCPs 1-20), may be compromised by disease-linked mutations. However, which of the several functions of CFH drives this self-surface selectivity remains unknown. To address this, we expressed human CFH mutants in Pichia pastoris We found that recombinant I62-CFH (protective against age-related macular degeneration) and V62-CFH functioned equivalently, matching or outperforming plasma-derived CFH, whereas R53H-CFH, linked to atypical hemolytic uremic syndrome (aHUS), was defective in C3bBb decay-accelerating activity (DAA) and factor I cofactor activity (CA)...
August 11, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28630239/an-unusual-case-of-escherichia-coli-o157-h7-infection-with-pseudomembranous-colitis-like-lesions-associated-with-haemolytic-uraemic-syndrome-and-neurological-sequelae
#9
James Kennedy, Lauren Simmonds, Robert Orme, Warren Doherty
A 75-year-old man was admitted with abdominal pain and fresh rectal bleeding. Significantly, he had no risk factors for Clostridium difficile infection. An abdominal CT demonstrated colonic thickening, and flexible sigmoidoscopy identified pseudomembranous colitis-like lesions. After initial treatment as C. difficile colitis, a stool sample revealed Escherichia coli O157:H7 infection. Antibiotic therapy was stopped due to the risk of lysis-mediated toxin release, but unfortunately, the patient continued to deteriorate...
June 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28621343/current-evidence-on-the-discontinuation-of-eculizumab-in-patients-with-atypical-haemolytic-uraemic-syndrome
#10
COMMENT
Manuel Macia, Fernando de Alvaro Moreno, Tina Dutt, Ingela Fehrman, Karine Hadaya, Christoph Gasteyger, Nils Heyne
Background. Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening disorder for which eculizumab is the only approved treatment. Life-long treatment is indicated; however, eculizumab discontinuation has been reported. Methods. Unpublished authors' cases and published cases of eculizumab discontinuation are reviewed. We also report eculizumab discontinuation data from five clinical trials, plus long-term extensions and the global aHUS Registry. Results. Of six unpublished authors' cases, four patients had a subsequent thrombotic microangiopathy (TMA) manifestation within 12 months of discontinuation...
June 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28597831/ongoing-haemolytic-uraemic-syndrome-hus-outbreak-caused-by-sorbitol-fermenting-sf-shiga-toxin-producing-escherichia-coli-stec-o157-germany-december-2016-to-may-2017
#11
Sabine Vygen-Bonnet, Bettina Rosner, Hendrik Wilking, Angelika Fruth, Rita Prager, Annelene Kossow, Christina Lang, Sandra Simon, Juliane Seidel, Mirko Faber, Anika Schielke, Kai Michaelis, Alexandra Holzer, Rolf Kamphausen, Daniela Kalhöfer, Sebastian Thole, Alexander Mellmann, Antje Flieger, Klaus Stark
We report an ongoing, protracted and geographically dispersed outbreak of haemolytic uraemic syndrome (HUS) and gastroenteritis in Germany, involving 30 cases since December 2016. The outbreak was caused by the sorbitol-fermenting immotile variant of Shiga toxin-producing (STEC) Escherichia coli O157. Molecular typing revealed close relatedness between isolates from 14 cases. One HUS patient died. Results of a case-control study suggest packaged minced meat as the most likely food vehicle. Food safety investigations are ongoing...
May 25, 2017: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/28559930/comparison-of-clinical-and-immunological-findings-in-gnotobiotic-piglets-infected-with-escherichia-coli-o104-h4-outbreak-strain-and-ehec-o157-h7
#12
Bettina Wöchtl, Florian Gunzer, Wilhelm Gerner, Hagen Gasse, Michaela Koch, Zoltán Bagó, Martin Ganter, Herbert Weissenböck, Nora Dinhopl, Sina M Coldewey, Alexandra von Altrock, Karl-Heinz Waldmann, Armin Saalmüller, Kurt Zimmermann, Jörg Steinmann, Jan Kehrmann, Ludger Klein-Hitpass, Jochen Blom, Ralf Ehricht, Ines Engelmann, Isabel Hennig-Pauka
BACKGROUND: Shiga toxin (Stx) producing Escherichia coli (E. coli) (STEC) is the most frequent cause of diarrhoea-positive haemolytic uraemic syndrome (D + HUS) in humans. In 2011, a huge outbreak with an STEC O104:H4 strain in Germany highlighted the limited possibilities for causative treatment of this syndrome. The responsible STEC strain was found to combine Stx production with adherence mechanisms normally found in enteroaggregative E. coli (EAEC). Pathotypes of E. coli evolve and can exhibit different adhesion mechanisms...
2017: Gut Pathogens
https://www.readbyqxmd.com/read/28500363/diffusion-weighted-imaging-of-the-kidneys-in-haemolytic-uraemic-syndrome
#13
Jochen Herrmann, Ulrich Wenzel, Stephanie Galler, Bjoern P Schoennagel, Jasmin D Busch, Magdalini Tozakidou, Kay U Petersen, Michaela Joekel, Peter Bannas, Jin Yamamura, Michael Groth, Gerhard Adam, Christian R Habermann
OBJECTIVES: To evaluate the kidneys of patients with haemolytic uraemic syndrome (HUS) using diffusion-weighted imaging (DWI) and Doppler ultrasound (US) compared with healthy controls. MATERIALS AND METHODS: Fifteen patients (mean age 33.3 years; three male; 12 female) with diarrhoea-positive HUS and 15 healthy volunteers were prospectively evaluated with DWI and Doppler US. A total apparent diffusion coefficient (ADCTOT), and ADCs predominantly reflecting microperfusion (ADCLOW) and diffusion (ADCHIGH) were calculated...
May 12, 2017: European Radiology
https://www.readbyqxmd.com/read/28446488/eculizumab-in-a-child-with-atypical-haemolytic-uraemic-syndrome-and-haemophagocytic-lymphohistiocytosis-triggered-by-cytomegalovirus-infection
#14
Gloria M Fraga-Rodriguez, Sonia Brió-Sanagustin, Eulalia Turón-Viñas, Bradley P Dixon, Eduardo Carreras-González
We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration.Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28442312/expert-statements-on-the-standard-of-care-in-critically-ill-adult-patients-with-atypical-hemolytic-uremic-syndrome
#15
REVIEW
Elie Azoulay, Paul Knoebl, José Garnacho-Montero, Katerina Rusinova, Gennadii Galstian, Philippe Eggimann, Fekri Abroug, Dominique Benoit, Michael von Bergwelt-Baildon, Julia Wendon, Marie Scully
A typical hemolytic uremic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP) and other causes or conditions with thrombotic microangiopathy (TMA), such as disseminated intravascular coagulation or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarize available data on the diagnosis and treatment strategies of aHUS in the ICU to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU...
August 2017: Chest
https://www.readbyqxmd.com/read/28426384/a-2-year-old-boy-with-circulatory-failure-owing-to-streptococcal-toxic-shock-syndrome-case-report
#16
Werner Keenswijk, Johan Vande Walle
A 2-year-old boy presented with severe hypotension and acute kidney injury after a prodrome of non-bloody diarrhoea and fever in the preceding 3 days. He had a mild Ebstein cardiac anomaly but otherwise a normal past history and growth. On examination, he looked ill, his temperature was 37.5 °C, circulation was poor, and there were several purpuric lesions on the face, hands and scrotum. Haemoglobin was 7.8 g/dL (11-14), total white cell count 27 × 10(9)/L, platelets 62 × 10(9)/L, blood urea nitrogen 20...
April 20, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28407128/the-bittersweet-taste-of-tubulo-interstitial-glycans
#17
REVIEW
Ditmer T Talsma, Mohamed R Daha, Jacob van den Born
Recently, interesting work was published by Farrar et al. [1] showing the interaction of fucosylated glycoproteins on stressed tubular epithelial cells with collectin-11 leading to complement activation via the lectin route of complement. This elegant work stimulated us to evaluate the dark side (bittersweet taste) of tubulo-interstitial glycans in kidney tissue damage. As will be discussed, glycans not only initiate tubular complement activation but also orchestrate tubulo-interstitial leucocyte recruitment and growth factor responses...
April 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28395633/haemolytic-uraemic-syndrome
#18
Doreen Crawford
Although haemolytic uraemic syndrome is rare, it is not uncommon. Here is an overview of the syndrome, with advice on signs and symptoms and how to care for children and young people.
April 11, 2017: Nursing Children and Young People
https://www.readbyqxmd.com/read/28391537/virulence-factors-of-shiga-toxin-producing-escherichia-coli-and-the-risk-of-developing-haemolytic-uraemic-syndrome-in-norway-1992-2013
#19
U Naseer, I Løbersli, M Hindrum, T Bruvik, L T Brandal
Shiga toxin-producing Escherichia coli (STEC) may cause haemolytic uraemic syndrome (HUS). Age ≤5 years and presence of stx2a and eae are risk factors for the development of HUS. In this study, we investigated STEC isolates for the presence of adhesins, toxins and molecular risk assessment (MRA) factors to identify virulence genes associated with HUS development. We included non-duplicate isolates from all STEC infections (n = 340, HUS = 32) reported to the Norwegian National Reference Laboratory (NRL) for Enteropathogenic Bacteria from 1992 to 2013...
April 8, 2017: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/28391343/cost-effectiveness-of-eculizumab-treatment-after-kidney-transplantation-in-patients-with-atypical-haemolytic-uraemic-syndrome
#20
Jan A J G van den Brand, Jacobien C Verhave, Eddy M Adang, Jack F M Wetzels
Background: Kidney transplantation in patients with atypical haemolytic uraemic syndrome (aHUS) is frequently complicated by recurrence of aHUS, often resulting in graft loss. Eculizumab prophylaxis prevents recurrence, improving graft survival. An alternative treatment strategy has been proposed where eculizumab is administered upon recurrence. We combined available evidence and performed a cost-effectiveness analysis of these competing strategies. Methods: A cost-effectiveness analysis using a decision analytical approach with Markov chain analyses was used to compare alternatives for aHUS patients with end-stage renal disease (ESRD): (i) dialysis treatment, (ii) kidney transplantation, (iii) kidney transplantation with eculizumab therapy upon recurrence of aHUS, (iv) kidney transplantation with eculizumab induction consisting of 12 months of prophylaxis and (v) kidney transplantation with lifelong eculizumab prophylaxis...
January 1, 2017: Nephrology, Dialysis, Transplantation
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