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Hemolytic uremic syndrome

Franz Schaefer, Gianluigi Ardissino, Gema Ariceta, Fadi Fakhouri, Marie Scully, Nicole Isbel, Åsa Lommelé, Varant Kupelian, Christoph Gasteyger, Larry A Greenbaum, Sally Johnson, Masayo Ogawa, Christoph Licht, Johan Vande Walle, Véronique Frémeaux-Bacchi
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, life-threatening disease. The Global aHUS Registry collects real-world data on the natural history of the disease. Here we characterize end-stage renal disease (ESRD)-free survival, the rate of thrombotic microangiopathy, organ involvement and the genetic background of 851 patients in the registry, prior to eculizumab treatment. A sex-specific difference was apparent according to age at initial disease onset as the ratio of males to females was 1...
June 12, 2018: Kidney International
Katarzyna Lipiec, Piotr Adamczyk, Elżbieta Świętochowska, Katarzyna Ziora, Maria Szczepańska
BACKGROUND: Hemolytic-uremic syndrome (HUS) is a form of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). From a clinical point of view, it is important to search for markers that allow for early identification of patients at risk of a poor prognosis. OBJECTIVES: The study evaluated the serum and urine levels of liver-type fatty acid binding protein (L-FABP) and interleukin 6 (IL-6). MATERIAL AND METHODS: The study was conducted in 29 children with a history of HUS...
June 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Sarah Luna, Vikram Krishnasamy, Louise Saw, Lori Smith, Jennifer Wagner, Jenna Weigand, Mackenzie Tewell, Marilee Kellis, Roumen Penev, Laine McCullough, Jeffrey Eason, Keegan McCaffrey, Cindy Burnett, Kelly Oakeson, Melissa Dimond, Allyn Nakashima, Deidre Barlow, Anna Scherzer, Melanie Sarino, Morgan Schroeder, Rashida Hassan, Colin Basler, Matthew Wise, Laura Gieraltowski
On June 26, 2017, a hospital in southern Utah notified the Utah Department of Health of Shiga toxin-producing Escherichia coli (STEC) O157:H7 infections in two children from a small community on the Arizona-Utah border. Both children developed hemolytic uremic syndrome, characterized by hemolytic anemia, acute kidney failure, and thrombocytopenia and died within a few days of illness onset. Over the next few days, several more STEC-associated illnesses were reported in residents of the community. A joint investigation by local and state health agencies from Arizona and Utah and CDC was initiated to identify the outbreak source and prevent additional cases; a total of 12 cases were identified, including the two children who died...
June 15, 2018: MMWR. Morbidity and Mortality Weekly Report
Megan Clancy, Ryan McGhan, Jeremy Gitomer, Ana Marie Inocencio, Christina Aldrich, Roberto Iaderosa, Ryan Stevens
PURPOSE: A case report of fatal disseminated cryptococcosis in a patient treated with eculizumab is presented along with a review of literature suggesting a possible etiologic mechanism. SUMMARY: A 23-year-old man with a history of minimal change nephrotic syndrome was hospitalized for acute kidney injury and abdominal pain and swelling. He was found to have disseminated pneumococcal disease, including peritonitis, bacteremia, and pulmonic endocarditis. The patient developed evidence of microangiopathic hemolytic anemia, leading to a diagnosis of atypical hemolytic uremic syndrome, and was started on eculizumab...
June 12, 2018: American Journal of Health-system Pharmacy: AJHP
M J Geerlings, E B Volokhina, E K de Jong, N van de Kar, M Pauper, C B Hoyng, L P van den Heuvel, A I den Hollander
Genetic alterations in the complement system have been linked to a variety of diseases, including atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and age-related macular degeneration (AMD). We performed sequence analysis of the complement genes CFH, CFI, and C3 in 866 aHUS/C3G and 697 AMD patients. In total we identified 505 low frequency alleles, representing 121 unique variants, of which 51 are novel. CFH contained the largest number of unique low frequency variants (n=64; 53%), followed by C3 (n=32; 26%) and CFI (n=25; 21%)...
June 11, 2018: Clinical Genetics
Meltem Polat, Selçuk Yüksel, Nuriye Ünal Şahin
Life-threatening and fatal meningococcal infections have occurred in patients treated with eculizumab. Herein we describe an 11-year-old boy with atypical hemolytic uremic syndrome treated with eculizumab who developed fatal meningococcemia due to Neisseria meningitidis serogroup Y 16 months after receiving two doses of meningococcal conjugate vaccine (MenACWY-D) while on oral penicillin prophylaxis. Clinicians need to maintain a high index of suspicion for invasive meningococcal disease in patients taking eculizumab treatment, regardless of meningococcal vaccination or antimicrobial prophylaxis status...
June 8, 2018: Human Vaccines & Immunotherapeutics
Martin Bommer, Manuela Wölfle-Guter, Stephan Bohl, Florian Kuchenbauer
BACKGROUND: Thrombotic microangiopathies are rare, life-threatening diseaseswhose care involves physicians from multiple specialties. The past five years haveseen major advances in our understanding of the pathophysiology, classification,and treatment of these conditions. Their timely diagnosis and prompt treatment cansave lives. METHODS: This review is based on pertinent articles published up to 17 December2017 that were retrieved by a selective search of the National Library of Medicine'sPubMed database employing the terms "thrombotic microangiopathy," "thromboticthrombocytopenic purpura," "hemolytic-uremic syndrome," "drug-induced TMA," and"EHEC-HUS...
May 11, 2018: Deutsches Ärzteblatt International
Thomas Douëllou, Wessam Galia, Stéphane Kerangart, Thierry Marchal, Nadège Milhau, Renaud Bastien, Marion Bouvier, Samuel Buff, Marie-Christine Montel, Delphine Sergentet-Thevenot
Enterohemorrhagic Escherichia coli (EHEC; E. coli ) are food-borne agents associated with gastroenteritis, enterocolitis, bloody diarrhea and the hemolytic-uremic syndrome (HUS). Bovine milk glycans have been shown to contain oligosaccharides which are similar to host epithelial cell receptors and can therefore prevent bacterial adhesion. This study aimed to describe interactions between EHEC O157:H7 EDL933 and O26:H11 21765 and milk fat globules (MFGs) in raw milk and raw milk cheese, and the impact of MFGs on EHEC strains adhesion to the intestinal tract in vitro and in vivo ...
2018: Frontiers in Microbiology
György Reusz
Complement is one of the most archaic parts of the innate immune system, which enhances the ability of antibodies and phagocytic cells to clear cell debris, and microorganisms. The complement system promotes inflammation and attacks the pathogen's plasma membrane. Malfunction of the system may lead to the development of autoimmunity or uncontrolled infections. Further, dysregulation of the tightly controlled complement activation process may lead to thrombotic microangiopathies with consequent multiorgan involvement...
June 2018: Orvosi Hetilap
Yoshiyasu Ueda, Takashi Miwa, Damodar Gullipalli, Sayaka Sato, Daisuke Ito, Hangsoo Kim, Matthew Palmer, Wen-Chao Song
Background Properdin (P) is a positive regulator of the alternative pathway of complement activation. Although P inhibition is expected and has been shown to ameliorate the alternative pathway of complement-mediated tissue injury in several disease models, it unexpectedly exacerbated renal injury in a murine model of C3 glomerulopathy. The role of P in atypical hemolytic uremic syndrome (aHUS) is uncertain. Methods We blocked P function by genetic deletion or mAb-mediated inhibition in mice carrying a factor H (FH) point mutation, W1206R (FHR/R ), that causes aHUS and systemic thrombophilia with high mortality...
June 1, 2018: Journal of the American Society of Nephrology: JASN
Daphne S Say, Trevor Starnes, Karen Matsukuma, Stephanie Nguyen
No abstract text is available yet for this article.
May 30, 2018: Journal of Pediatric Gastroenterology and Nutrition
Mihaela Balgradean, Anca Croitoru, Eugene Leibovitz
BACKGROUND AND AIMS: To describe the epidemiologic, clinical, microbiological, therapeutic and outcome characteristics of a HUS outbreak occurring in southern Romania from 2015 to 2016. METHODS: We retrospectively collected data from the medical records of all HUS cases hospitalized at the pediatric nephrology department of Marie Curie Children's Hospital of Bucharest, Romania. RESULTS: There were 32 HUS cases (19 girls/13 boys, 87.6% <2 years), all associated with diarrhea (bloody in 13, 40...
May 8, 2018: Pediatrics and Neonatology
Luisina Martorelli, Nicolás Garimano, Gabriela A Fiorentino, Daniel A Vilte, Sergio G Garbaccio, Stefanie A Barth, Christian Menge, Cristina Ibarra, Marina S Palermo, Angel Cataldi
Escherichia coli O157:H7 is a zoonotic pathogen of global importance and the serotype of Shiga toxin-producing E.coli (STEC) most frequently associated with Hemolytic Uremic Syndrome (HUS) in humans. The main STEC reservoir is cattle. Vaccination of calves with the carboxy-terminal fraction of Intimin γ (IntC280) and EspB can reduce E.coli O157:H7 fecal shedding after experimental challenge. Shiga toxin (Stx) exerts local immunosuppressive effects in the bovine intestine and Stx2B fused to Brucella lumazine synthase (BLS-Stx2B) induces Stx2-neutralizing antibodies...
May 25, 2018: Vaccine
Bradley P Dixon, Ralph A Gruppo
Atypical hemolytic uremic syndrome is a rare life-threatening disease of unregulated complement activation. Untreated, the prognosis is generally poor; more than one-half of patients die or develop end-stage renal disease within 1 year. Atypical hemolytic uremic syndrome is characterized by thrombotic microangiopathy with evidence of hemolysis, thrombocytopenia, and renal impairment. This systemic disease affects the kidneys, brain, heart, lungs, gastrointestinal tract, pancreas, and skin. Acquired and genetic abnormalities of complement regulation may be identified in approximately 70% of patients...
June 2018: Pediatric Clinics of North America
Joseph L Alge, Scott E Wenderfer
No abstract text is available yet for this article.
June 2018: Journal of Pediatrics
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...
January 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Shambhuprasad K Togarsimalemath, Ali Si-Mohammed, Mamta Puraswani, Aarti Gupta, Astrid Vabret, Sandrine Liguori, Patricia Mariani-Kurkdjian, Arvind Bagga, Marie-Agnes Dragon-Durey
BACKGROUND: Prodromal symptoms are frequently reported in the atypical form of Hemolytic uremic syndrome (aHUS) suggesting implication of infectious triggers. Some pathogens may also play a role in the mechanisms of production of autoantibody directed against Factor H (FH), a complement regulator, leading to aHUS. METHODS: The presence of 15 gastrointestinal (GI) pathogens was investigated by using xTAG-based multiplex PCR techniques on stools collected at the acute phase in a cohort of Indian HUS children classified according to the presence or absence of anti-FH autoantibodies...
May 23, 2018: Pediatric Research
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
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
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
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