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haemolytic uraemic syndrome

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https://www.readbyqxmd.com/read/27905001/guidelines-for-diagnosis-and-management-of-the-cobalamin-related-remethylation-disorders-cblc-cbld-cble-cblf-cblg-cblj-and-mthfr-deficiency
#1
Martina Huemer, Daria Diodato, Bernd Schwahn, Manuel Schiff, Anabela Bandeira, Jean-Francois Benoist, Alberto Burlina, Roberto Cerone, Maria L Couce, Angeles Garcia-Cazorla, Giancarlo la Marca, Elisabetta Pasquini, Laura Vilarinho, James D Weisfeld-Adams, Viktor Kožich, Henk Blom, Matthias R Baumgartner, Carlo Dionisi-Vici
BACKGROUND: Remethylation defects are rare inherited disorders in which impaired remethylation of homocysteine to methionine leads to accumulation of homocysteine and perturbation of numerous methylation reactions. OBJECTIVE: To summarise clinical and biochemical characteristics of these severe disorders and to provide guidelines on diagnosis and management. DATA SOURCES: Review, evaluation and discussion of the medical literature (Medline, Cochrane databases) by a panel of experts on these rare diseases following the GRADE approach...
November 30, 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27891338/non-o157-h7-shiga-toxin-producing-diarrhoeagenic-escherichia-coli-stec-in-southern-india-a-tinderbox-for-starting-epidemic
#2
Shashank Purwar, Subrana Roy, Sharada Metgud
INTRODUCTION: Outbreaks due to non-O157:H7 Shiga toxin producing Escherichia coli (STEC) resulting in Haemolytic Uraemic Syndrome (HUS) have garnered much attention because of associated mortality transcending across continents and also because diarrhoea due to E.coli itself is rare in developed countries. The actual incidence of non-O157:H7 STEC in sporadic acute diarrhoea is not fully elucidated, both in developing as well as in developed countries. Due to larger extent of faecal-oral transmission in developing countries it is prudent to look for non-O157: H7 STEC in such epidemiological settings because of very high potential to spread across larger geographical regions and cause life threatening illness...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27871301/an-innovative-and-collaborative-partnership-between-patients-with-rare-disease-and-industry-supported-registries-the-global-ahus-registry
#3
REVIEW
Len Woodward, Sally Johnson, Johan Vande Walle, Joran Beck, Christoph Gasteyger, Christoph Licht, Gema Ariceta
BACKGROUND: Patients are becoming increasingly involved in research which can promote innovation through novel ideas, support patient-centred actions, and facilitate drug development. For rare diseases, registries that collect data from patients can increase knowledge of the disease's natural history, evaluate clinical therapies, monitor drug safety, and measure quality of care. The active participation of patients is expected to optimise rare-disease management and improve patient outcomes...
November 21, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27784011/decreased-neutrophil-extracellular-trap-degradation-in-shiga-toxin-associated-haemolytic-uraemic-syndrome
#4
Jonatan Leffler, Zoltán Prohászka, Bálint Mikes, György Sinkovits, Katarzyna Ciacma, Péter Farkas, Marienn Réti, Kata Kelen, György S Reusz, Attila J Szabó, Myriam Martin, Anna M Blom
BACKGROUND: Neutrophil extracellular traps (NETs) can stimulate thrombosis, and their degradation is decreased in several autoimmune disorders. It was recently reported that some patients with haemolytic uraemic syndrome (HUS) also fail to degrade NETs and that neutrophils from Shiga toxin-associated HUS are primed to form NETs. METHOD: We used a well-characterized cohort of 74 thrombotic microangiopathy (TMA) patients, with a subset also providing follow-up samples, and 112 age-matched controls to investigate NET degradation and serum nuclease activity in TMA before, during and after treatment...
October 27, 2016: Journal of Innate Immunity
https://www.readbyqxmd.com/read/27723152/haemolytic-uraemic-syndrome
#5
Diana Karpman, Sebastian Loos, Ramesh Tati, Ida Arvidsson
Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains...
October 10, 2016: Journal of Internal Medicine
https://www.readbyqxmd.com/read/27684204/community-wide-outbreak-of-haemolytic-uraemic-syndrome-associated-with-shiga-toxin-2-producing-escherichia-coli-o26-h11-in-southern-italy-summer-2013
#6
Cinzia Germinario, Alfredo Caprioli, Mario Giordano, Maria Chironna, Maria Serena Gallone, Silvio Tafuri, Fabio Minelli, Antonella Maugliani, Valeria Michelacci, Luisa Santangelo, Onofrio Mongelli, Cosimo Montagna, Gaia Scavia
In summer 2013, an excess of paediatric cases of haemolytic uraemic syndrome (HUS) in a southern region of Italy prompted the investigation of a community-wide outbreak of Shiga toxin 2-producing Escherichia coli (STEC) O26:H11 infections. Case finding was based on testing patients with HUS or bloody diarrhoea for STEC infection by microbiological and serological methods. A case-control study was conducted to identify the source of the outbreak. STEC O26 infection was identified in 20 children (median age 17 months) with HUS, two of whom reported severe neurological sequelae...
September 22, 2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/27671993/steak-and-stupor-seizures-and-e-coli-o157-infection
#7
Sherri A Braksick, Jennifer M Martinez-Thompson, Eelco F M Wijdicks
Neurological complications of haemolytic uraemic syndrome (HUS) include altered states of consciousness, seizures, ischaemic stroke and encephalopathy. Adult-onset HUS is uncommon, and there is only a limited literature reporting neurological complications in this population. We report an adult with Shiga toxin-associated HUS complicated by focal-onset non-convulsive status epilepticus, who made a full neurological recovery.
September 26, 2016: Practical Neurology
https://www.readbyqxmd.com/read/27587606/atypical-haemolytic-uraemic-syndrome-and-pregnancy-outcome-with-ongoing-eculizumab
#8
Aude Servais, Nadège Devillard, Véronique Frémeaux-Bacchi, Aurélie Hummel, Laurent Salomon, Cécile Contin-Bordes, Hélène Gomer, Christophe Legendre, Yahsou Delmas
BACKGROUND: A therapeutic strategy based on complement blockade by eculizumab is widely used to treat atypical haemolytic uraemic syndrome (aHUS). Recent data are available on the administration of eculizumab during pregnancy in patients treated for paroxysmal nocturnal haemoglobinuria but there are very few data for aHUS patients. METHODS: We analysed the use of eculizumab for the treatment of aHUS during five pregnancies in three patients and studied an additional pregnancy without eculizumab...
September 1, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27468812/estimating-true-incidence-of-o157-and-non-o157-shiga-toxin-producing-escherichia-coli-illness-in-germany-based-on-notification-data-of-haemolytic-uraemic-syndrome
#9
A Kuehne, M Bouwknegt, A Havelaar, A Gilsdorf, P Hoyer, K Stark, D Werber
Shiga toxin-producing Escherichia coli (STEC) is an important cause of gastroenteritis (GE) and haemolytic uraemic syndrome (HUS). Incidence of STEC illness is largely underestimated in notification data, particularly of serogroups other than O157 ('non-O157'). Using HUS national notification data (2008-2012, excluding 2011), we modelled true annual incidence of STEC illness in Germany separately for O157 and non-O157 STEC, taking into account the groups' different probabilities of causing bloody diarrhoea and HUS, and the resulting difference in their under-ascertainment...
November 2016: Epidemiology and Infection
https://www.readbyqxmd.com/read/27452363/anti-complement-factor-h-associated-glomerulopathies
#10
REVIEW
Marie-Agnes Dragon Durey, Aditi Sinha, Shambhuprasad Kotresh Togarsimalemath, Arvind Bagga
Atypical haemolytic uraemic syndrome (aHUS), an important cause of acute kidney injury, is characterized by dysregulation of the complement pathway, frequent need for dialysis, and progression to end-stage renal disease. Autoantibodies against complement factor H (FH), the main plasma regulatory protein of the alternative pathway of the complement system, account for a considerable proportion of children with aHUS. The autoantibodies are usually associated with the occurrence of a homozygous deletion in the genes encoding the FH-related proteins FHR1 and FHR3...
September 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27433282/cancer-associated-thrombotic-microangiopathy
#11
REVIEW
K Govind Babu, Gita R Bhat
Cancer-associated thrombotic microangiopathy refers to a group of disorders characterised by microvascular thrombosis, thrombocytopenia, and ischaemic end-organ damage. Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura are the two major subtypes. It can be a manifestation of the malignancy itself or a complication of its therapy. The addition of several new drugs to the therapeutic armamentarium of cancer has brought to light several novel causative factors of this hitherto uncommon complication...
2016: Ecancermedicalscience
https://www.readbyqxmd.com/read/27387818/molecular-characterization-and-antimicrobial-resistance-of-stec-strains-isolated-from-healthy-cattle-in-2011-and-2013-in-spain
#12
A Cabal, M C Porrero, M L DE LA Cruz, J L Saez, C Barcena, G Lopez, C Gortazar, L Dominguez, J Alvarez
Prevention of Shiga toxin-producing Escherichia coli (STEC) foodborne outbreaks is hampered by its complex epidemiology. We assessed the distribution of virulence genes (VGs), main serogroups/serotypes for public health [haemolytic uraemic syndrome (HUS)-related], antimicrobial resistance (AMR) profiles and pulsed-field gel electrophoresis (PFGE) patterns in a collection of STEC isolates obtained from cattle hide (n = 149) and faecal (n = 406) samples collected during a national survey conducted in Spain in 2011 and 2013...
July 8, 2016: Epidemiology and Infection
https://www.readbyqxmd.com/read/27382949/an-unusual-case-of-haemolytic-uraemic-syndrome-following-endoscopic-retrograde-cholangiopancreatography-rapidly-improved-with-eculizumab
#13
O Taton, M Delhaye, P Stordeur, T Goodship, A Le Moine, A Massart
Atypical haemolytic uraemic syndrome (aHUS) is a rare but life-threatening complement system-related disorder, characterized by renal failure, non-immune haemolytic anaemia and thrombo-cytopenia. We report on a young woman who developed a pancreatitis-induced aHUS following a routine procedure of endoscopic retrograde cholangiopancreatography. The patient was successively treated by 2 plasma exchanges with fresh frozen plasma and eculizumab, a monoclonal antibody designed to block terminal complement activation...
April 2016: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/27374918/the-expanding-phenotypic-spectra-of-kidney-diseases-insights-from-genetic-studies
#14
REVIEW
Marijn F Stokman, Kirsten Y Renkema, Rachel H Giles, Franz Schaefer, Nine V A M Knoers, Albertien M van Eerde
Next-generation sequencing (NGS) has led to the identification of previously unrecognized phenotypes associated with classic kidney disease genes. In addition to improving diagnostics for genetically heterogeneous diseases and enabling a faster rate of gene discovery, NGS has enabled an expansion and redefinition of nephrogenetic disease categories. Findings from these studies raise the question of whether disease diagnoses should be made on clinical grounds, on genetic evidence or a combination thereof. Here, we discuss the major kidney disease-associated genes and gene categories for which NGS has expanded the phenotypic spectrum...
August 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27313274/chronic-peritoneal-dialysis-in-chinese-infants-and-children-younger-than-two-years
#15
Y H Chan, A Lt Ma, P C Tong, W M Lai, N Kc Tse
OBJECTIVE: To review the outcome for Chinese infants and young children on chronic peritoneal dialysis. METHODS: The Paediatric Nephrology Centre of Princess Margaret Hospital is the designated site offering chronic dialysis to children in Hong Kong. Medical records of children who started chronic peritoneal dialysis before the age of 2 years, from 1 July 1995 to 31 December 2013, were retrieved and retrospectively reviewed. RESULTS: Nine Chinese patients (male-to-female ratio, 3:6) were identified...
August 2016: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
https://www.readbyqxmd.com/read/27297133/an-outbreak-of-shiga-toxin-producing-escherichia-coli-serogroup-o157-linked-to-a-lamb-feeding-event
#16
S Rowell, C King, C Jenkins, T J Dallman, V Decraene, K Lamden, A Howard, C A Featherstone, P Cleary
Fifteen confirmed cases and 15 possible cases of Shiga toxin-producing Escherichia coli (STEC) O157 phage type 21/28 were linked to direct contact with lambs at a 'Lambing Live' event in the North West of England between 29 March and 21 April 2014. Twenty-one (70%) of the cases were female, 23 (77%) were children aged <16 years, of whom 14 (46%) were in the 0-5 years age group. Five children developed haemolytic uraemic syndrome. Multilocus variable number tandem repeat analysis (MLVA) profiles on 14 human cases were indistinguishable, and 6/10 animal isolates had a MLVA profile identical to the outbreak profile...
September 2016: Epidemiology and Infection
https://www.readbyqxmd.com/read/27268256/chromosomal-rearrangement-a-rare-cause-of-complement-factor-i-associated-atypical-haemolytic-uraemic-syndrome
#17
Patrick J Gleeson, Valerie Wilson, Thomas E Cox, Seema D Sharma, Kate Smith-Jackson, Lisa Strain, David Lappin, Teresa McHale, David Kavanagh, Timothy H J Goodship
Chromosomal rearrangements affecting the genes encoding complement factor H and the factor H related proteins have been described in aHUS patients. To date such disorders have not been described in other aHUS associated genes. We describe here a heterozygous 875,324bp deletion encompassing the gene (CFI) encoding complement factor I and ten other genes. The index case presented with aHUS and did not recover renal function. No abnormalities were detected on Sanger sequencing of CFI but a low factor I level led to a multiplex ligation-dependent probe amplification assay being undertaken...
October 2016: Immunobiology
https://www.readbyqxmd.com/read/27267919/autoantibodies-against-alternative-complement-pathway-proteins-in-renal-pathologies
#18
Pilar Nozal, Margarita López-Trascasa
Complement system activation plays an important role in several renal pathologies, including antibody-mediated glomerulonephritis, ischaemia-reperfusion injury of trasplanted kidneys or renal allograft rejection. Besides these conditions, alternative pathway abnormalities are directly involved in the pathogenesis of C3 glomerulopathies and atypical haemolytic uraemic syndrome. These abnormalities may be inherited or acquired, the latter as autoantibodies directed against the various components and regulators of the alternative complement pathway...
June 3, 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/27228393/diabetes-recurrence-after-haemolytic-uraemic-syndrome-outbreak-in-adelaide
#19
Alexia Sophie Peña, Amanda Graham, Varsha Rao, Paul Henning, Jennifer Couper
No abstract text is available yet for this article.
July 2016: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27190382/systemic-complement-activation-and-complement-gene-analysis-in-enterohaemorrhagic-escherichia-coli-associated-paediatric-haemolytic-uraemic-syndrome
#20
Thurid Ahlenstiel-Grunow, Svenja Hachmeister, Franz Christoph Bange, Cyrill Wehling, Michael Kirschfink, Carsten Bergmann, Lars Pape
BACKGROUND: In contrast to atypical haemolytic uraemic syndrome (aHUS), only single case reports and limited data have been published on systemic activation of the complement system and mutations in complement genes in paediatric enterohaemorrhagic Escherichia coli-induced HUS (EHEC-HUS). METHODS: Complement activation (CH50, APH50, C3d, sC5b-9) was analysed at four timepoints (Week 1, Week 2, Month 3 and Month 6 after primary diagnosis of HUS) in 25 children with EHEC-HUS...
July 2016: Nephrology, Dialysis, Transplantation
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