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haemolytic uraemic syndrome

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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
M Piastra, A Tempera, E Luca, E Buffone, C Cafforio, V Briganti, O Genovese, M Marano, D Rigante
Streptococcus pneumoniae sepsis has high morbidity, particularly if complicated by renal injury. Four patients with S. pneumonia invasive infections complicated by renal disorders are presented. The first case was an 18-month-old girl with pneumococcal empyema complicated by haemolytic uraemic (HUS) syndrome. She made a full recovery after mechanical ventilation, inotropic support and haemodiafiltration. The second was a 4-year-old boy who presented with acute post-infectious glomerulonephritis associated with bilateral pneumococcal pneumonia...
January 29, 2016: Paediatrics and International Child Health
Emilie Peron, Alina Zaharia, Lavinia Cipriana Zota, Ettore Severi, Otilia Mårdh, Codruta Usein, Mihaela Bălgrădean, Laura Espinosa, Josep Jansa, Gaia Scavia, Alexandru Rafila, Amalia Serban, Adriana Pistol
As at 29 February 2016, 15 cases of haemolytic uraemic syndrome with onset between 25 January and 22 February were reported among children between five and 38 months in Romania, and three of them died. Cases were mostly from southern Romania. Six cases tested positive for Escherichia coli O26 by serology. Fruits, vegetables, meat and dairy products were among the possible common food exposures. Investigations are ongoing in Romania to control the outbreak.
2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
Mari R Thomas, Susan Robinson, Marie A Scully
Differentiation between the thrombotic microangiopathies (TMAs) that present in pregnancy may be clinically challenging, but is critical to ensure correct management because of the impact on fetal and maternal outcomes. Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are medical/obstetric emergencies that require specialist input, both at the time of acute diagnosis and follow-up in subsequent pregnancies. Features of preeclampsia and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) may precede or be present in evolving TTP or aHUS...
June 2016: British Journal of Haematology
Ángel Augusto Pérez-Calatayud, Jesús Carlos Briones-Garduño, Mercedes Del Pilar Álvarez-Goris, Ricardo Sánchez Zamora, Angélica A Torres Aguilar, Rosa Elba Mendoza-Mórales
Atypical haemolytic uraemic syndrome is one of the main variants of thrombotic microangiopathy, and is characterized by excessive complement activation in the microvasculature. It is also characterised by the clinical triad; non-immune haemolytic anaemia, thrombocytopenia, and acute renal failure. In addition, 60% of patients have mutations in the genes encoding complement regulators (factor H, factor I, membrane cofactor proteins, and thrombomodulin), activators (factor B and C3), as well as autoantibodies against factor H...
July 2016: Cirugia y Cirujanos
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