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
Association of a Factor H Mutation With Hemolytic Uremic Syndrome Following a Diarrheal Illness
Matthew Edey,Matthew Edey,Paul A. Mead,Rebecca E. Saunders,Lisa Strain,Stephen J. Perkins,Timothy H.J. Goodship,Timothy H.J. Goodship,N. Suren Kanagasundaram +8 more
TL;DR: In this article, a case of HUS after a diarrheal illness in which the patient did not recover renal function in the long term was discovered, a novel mutation in exon 23 of the factor H gene was discovered.
Journal ArticleDOI
Structural and functional characterization of the product of disease-related factor H gene conversion.
Andrew P. Herbert,David J. Kavanagh,Conny M. Johansson,Hugh P. Morgan,Baerbel S. Blaum,Jonathan P. Hannan,Paul N. Barlow,Dušan Uhrín +7 more
TL;DR: It is shown that FH19-20 mutants containing Leu1191 self-associated on a heparin-coated surface to a higher degree than on surfaces coated with dermatan or chondroitin sulfates, and potentially disease-related functional distinctions between mutants, and between FH and FH-related 1, may manifest in the presence of specific glycosaminoglycans.
Journal ArticleDOI
Does complement factor B have a role in the pathogenesis of atypical HUS
David J. Kavanagh,Elizabeth J. Kemp,Anna Richards,Rachel M. Burgess,Elizabeth Mayland,Judith Goodship,Timothy H.J. Goodship +6 more
TL;DR: There is no evidence that fB has a major role in the pathogenesis of aHUS, and no statistical difference between controls and aH US patients in either BF allele or haplotype frequency.
Journal ArticleDOI
Triggering of atypical hemolytic uremic syndrome by influenza A (H1N1).
Diogo Bento,Joana Mapril,Carla Rocha,Kevin J. Marchbank,David J. Kavanagh,Dawn Barge,Lisa Strain,Timothy H.J. Goodship,Carlos Meneses-Oliveira +8 more
TL;DR: A case of atypical hemolytic uremic syndrome triggered by influenza A (H1N1) in a 17-year-old boy with a mutation in the gene (CD46) encoding the transmembrane complement regulator membrane cofactor protein is reported.
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