Atypical Hemolytic Uremic Syndrome
TLDR
HUS is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure that predicts the prognosis both in native kidneys and after renal transplantation.About:
This article is published in Seminars in Nephrology.The article was published on 2013-11-01 and is currently open access. It has received 258 citations till now. The article focuses on the topics: Atypical hemolytic uremic syndrome & Eculizumab.read more
Citations
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Journal ArticleDOI
Atypical hemolytic uremic syndrome
TL;DR: Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care.
Journal ArticleDOI
Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference
Timothy H.J. Goodship,H. Terence Cook,Fadi Fakhouri,Fernando C. Fervenza,Véronique Frémeaux-Bacchi,David J. Kavanagh,Carla M. Nester,Marina Noris,Matthew C. Pickering,Santiago Rodríguez de Córdoba,Lubka T. Roumenina,Lubka T. Roumenina,Lubka T. Roumenina,Sanjeev Sethi,Richard J.H. Smith,Charlie E. Alpers,Gerald B. Appel,Gianluigi Ardissino,Gema Ariceta,Mustafa Arici,Arvind Bagga,Ingeborg M. Bajema,Miguel Blasco,Linda Burke,Thomas D. Cairns,Mireya Carratala,Vivette D. D'Agati,Mohamed R. Daha,An S. De Vriese,Marie-Agnès Dragon-Durey,Agnes B. Fogo,Miriam Galbusera,Daniel P. Gale,Hermann Haller,Sally Johnson,Mihály Józsi,Diana Karpman,Lynne D. Lanning,Moglie Le Quintrec,Christoph Licht,Chantal Loirat,Francisco Monfort,B. Paul Morgan,Laure Hélène Noël,Michelle M. O’Shaughnessy,Marion Rabant,Eric Rondeau,Piero Ruggenenti,Neil S. Sheerin,Jenna L.H. Smith,Fabrizio Spoleti,Joshua M. Thurman,Nicole C. A. J. van de Kar,Marina Vivarelli,Peter F. Zipfel +54 more
TL;DR: Recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options, and knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.
Journal ArticleDOI
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Chantal Loirat,Fadi Fakhouri,Gema Ariceta,Nesrin Besbas,Martin Bitzan,Anna Bjerre,Rosanna Coppo,Francesco Emma,Sally Johnson,Diana Karpman,Daniel Landau,Craig B. Langman,Anne Laure Lapeyraque,Christoph Licht,Carla M. Nester,Carmine Pecoraro,Magdalena Riedl,Nicole C. A. J. van de Kar,Johan Vande Walle,Marina Vivarelli,Véronique Frémeaux-Bacchi +20 more
TL;DR: The need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations is discussed.
Journal ArticleDOI
Complement, a target for therapy in inflammatory and degenerative diseases.
TL;DR: The history, current landscape and future directions for anti-complement therapies are described, which include infectious, inflammatory, degenerative, traumatic and neoplastic disorders.
Journal ArticleDOI
Dynamics of complement activation in aHUS and how to monitor eculizumab therapy
Marina Noris,Miriam Galbusera,Sara Gastoldi,Paolo Macor,Federica Banterla,Elena Bresin,Claudio Tripodo,Serena Bettoni,Roberta Donadelli,Elisabetta Valoti,Francesco Tedesco,Alessandro Amore,Rosanna Coppo,Piero Ruggenenti,Eliana Gotti,Giuseppe Remuzzi +15 more
TL;DR: Results point to efficient complement inhibition on endothelium for aHUS treatment, including C5b-9 endothelial deposits, which might help monitor eculizumab effectiveness, avoid drug overexposure, and save money considering the extremely high cost of the drug.
References
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Journal ArticleDOI
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome.
Jessica Caprioli,Marina Noris,Simona Brioschi,Gaia Pianetti,Federica Castelletti,Paola Bettinaglio,Caterina Mele,Elena Bresin,Linda Cassis,Sara Gamba,Francesca Porrati,Sara Bucchioni,Giuseppe Monteferrante,Celia J. Fang,M K Liszewski,David J. Kavanagh,John P. Atkinson,Giuseppe Remuzzi +17 more
TL;DR: The presentation, the response to therapy, and the outcome of the disease are influenced by the genotype, which will translate into improved management and therapy of patients and will provide the way to design tailored treatments.
Journal ArticleDOI
Genetics and Outcome of Atypical Hemolytic Uremic Syndrome: A Nationwide French Series Comparing Children and Adults
Véronique Frémeaux-Bacchi,Fadi Fakhouri,Arnaud Garnier,Frank Bienaimé,Marie-Agnès Dragon-Durey,Stéphanie Ngo,Bruno Moulin,Aude Servais,François Provôt,Lionel Rostaing,Stéphane Burtey,Patrick Niaudet,Georges Deschênes,Yvon Lebranchu,Julien Zuber,Chantal Loirat +15 more
TL;DR: Mortality rate was higher in children than adults with aHUS, but renal prognosis was worse in adults than children, and in children, the prognosis strongly depends on the genetic background.
Journal ArticleDOI
Atypical hemolytic uremic syndrome
TL;DR: Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care.
Journal ArticleDOI
Genetic studies into inherited and sporadic hemolytic uremic syndrome
Paul Warwicker,Timothy H.J. Goodship,Timothy H.J. Goodship,Timothy H.J. Goodship,Rosemary L. Donne,Rosemary L. Donne,Rosemary L. Donne,Yves Pirson,Yves Pirson,Yves Pirson,A. J. Nicholls,A. J. Nicholls,A. J. Nicholls,Roy Ward,Roy Ward,Roy Ward,Peter D Turnpenny,Peter D Turnpenny,Peter D Turnpenny,Judith A. Goodship,Judith A. Goodship,Judith A. Goodship +21 more
TL;DR: It is postulate that abnormalities of factor H may be involved in the etiology of HUS.
Journal ArticleDOI
Thrombomodulin mutations in atypical hemolytic-uremic syndrome.
Mieke Delvaeye,Marina Noris,Astrid De Vriese,Charles T. Esmon,Naomi L. Esmon,Gary L. Ferrell,Jurgen Del-Favero,Stéphane Plaisance,Bart Claes,Diether Lambrechts,Carla Zoja,Giuseppe Remuzzi,Edward M. Conway +12 more
TL;DR: In this paper, the role of thrombomodulin, an endothelial glycoprotein with anticoagulant, antiinflammatory, and cytoprotective properties, in atypical hemolytic-uremic syndrome was investigated.
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