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
Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency
Mihály Józsi,Christoph Licht,Stefanie Strobel,Svante L. H. Zipfel,Heiko Richter,Stefan Heinen,Peter F. Zipfel,Peter F. Zipfel,Christine Skerka +8 more
TL;DR: A novel subgroup of aHUS is defined, termed DEAP HUS (deficiency of CFHR proteins and CFH autoantibody positive) that is characterized by a combination of genetic and acquired factors.
Journal ArticleDOI
Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome
Iain Moore,Lisa Strain,Isabel Y. Pappworth,David J. Kavanagh,Paul N. Barlow,Andrew P. Herbert,Christoph Q. Schmidt,Scott J. Staniforth,Lucy V. Holmes,Roy Ward,Lynn Morgan,Timothy H.J. Goodship,Kevin J. Marchbank +12 more
TL;DR: The prevalence of factor H autoantibodies in the Newcastle cohort of aHUS patients is examined, whether such patients have additional susceptibility factors and/or mutations in the genes encoding complement regulator/activators are examined, and whether the presence of such autoantIBodies is always associated with deficiency offactor H-related proteins 1 and 3.
Journal ArticleDOI
Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome
Véronique Frémeaux-Bacchi,Marie-Agnès Dragon-Durey,Jacques Blouin,C Vigneau,Dirk Kuypers,Bernard Boudailliez,Chantal Loirat,Eric Rondeau,Wolf H. Fridman +8 more
TL;DR: Two complement alternative pathway proteins, factor H (FH) and recently membrane cofactor protein (CD46; MCP) have been identified as fostering the development of atypical HUS.
Journal ArticleDOI
Eculizumab for atypical hemolytic-uremic syndrome.
Jens Nürnberger,Thomas Philipp,Oliver Witzke,Anabelle Opazo Saez,Udo Vester,Hideo A. Baba,Andreas Kribben,Lothar Bernd Zimmerhackl,Andreas R. Janecke,Mato Nagel,Michael Kirschfink +10 more
TL;DR: A case of atypical hemolytic–uremic syndrome is reported that was successfully treated with eculizumab, a humanized monoclonal antibody that blocks complement activity by cleavage of the complement protein C5, thereby preventing the generation of the inflammatory.
Journal ArticleDOI
Factor H Mutations in Hemolytic Uremic Syndrome Cluster in Exons 18–20, a Domain Important for Host Cell Recognition
Anna Richards,M Buddles,Rosemary L. Donne,Bernard S. Kaplan,Edwin E. Kirk,Michael Venning,Christian Tielemans,Judith A. Goodship,Tim T. Goodship +8 more
TL;DR: Mutation screening of the FH gene in 19 familial and 31 sporadic patients with FH demonstrates that familial HUS is likely to be a heterogeneous condition.
Related Papers (5)
Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype
Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic–Uremic Syndrome
Ch. Legendre,Christoph Licht,Petra Muus,Laurence Greenbaum,Sunil Babu,C. Bedrosian,C. Bingham,David J. Cohen,Y. Delmas,Kenneth W. Douglas,Frank Eitner,Thorsten Feldkamp,Denis Fouque,Richard R. Furman,Osama Gaber,Maria Herthelius,Maryvonne Hourmant,Diana Karpman,Yvon Lebranchu,Christophe Mariat,Jan Menne,Bruno Moulin,Jens Nürnberger,Masayo Ogawa,Giuseppe Remuzzi,T. Richard,Rebecca Sberro-Soussan,B. Severino,Neil S. Sheerin,Antonella Trivelli,L.B. Zimmerhackl,Timothy H.J. Goodship,Chantal Loirat +32 more