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
Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains
Matthew C. Pickering,Elena Goicoechea de Jorge,Rubén Martínez-Barricarte,Sergio Recalde,Alfredo García-Layana,Kirsten L. Rose,Jill Moss,Mark Walport,H. Terence Cook,Santiago Rodríguez de Córdoba,Marina Botto +10 more
TL;DR: These animals represent the first model of aHUS and provide in vivo evidence that effective plasma C3 regulation and the defective control of complement activation on renal endothelium are the critical events in the molecular pathogenesis of FH-associated aH US.
Journal ArticleDOI
Eculizumab for congenital atypical hemolytic-uremic syndrome.
Ralph A Gruppo,Russell P. Rother +1 more
TL;DR: A patient with congenital relapsing atypical hemolytic–uremic syndrome who was unresponsive to plasma therapy but had a response to eculizumab, a humanized monoclonal antibody against terminal complement protein C5 is reported on.
Journal ArticleDOI
A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders.
Nesrin Besbas,Diana Karpman,Daniel Landau,Chantal Loirat,Willem Proesmans,Giuseppe Remuzzi,G. Rizzoni,C.M. Taylor,N.C.A.J. van de Kar,L.B. Zimmerhackl +9 more
TL;DR: A classification that accommodates both a current understanding of causation and clinical association in cases for whom cause of disease is unclear is proposed and is expected to increase the proportion of cases in whom a level 1 etiological diagnosis is confirmed.
Journal ArticleDOI
Anti–factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome
Mihály Józsi,Stefanie Strobel,Hans-Martin Dahse,Wei-shih Liu,Peter F. Hoyer,Martin Oppermann,Christine Skerka,Peter F. Zipfel,Peter F. Zipfel +8 more
TL;DR: It is suggested that aHUS-associated FH autoantibodies mimic the effect of C-terminal FH mutations, as they inhibit the regulatory function of FH at cell surfaces by blocking its C-Terminal recognition region.
Journal ArticleDOI
An Observational Study of Anaemia Management in Patients with NHL Receiving CHOP-14 or -21 (With or Without Rituximab)
Gregor Verhoef,Hans Erik Johnsen,Francesca Gaia Rossi,Antonio Salar,Lisa Hamilton,W. Marieke Schoonen,B. Pujol,Ulrich Dührsen +7 more
TL;DR: European patients with diffuse large-B-cell lymphoma (DLBCL) receiving CHOP-14 or -21 may experience treatment-related anaemia that can be managed with ESAs that provides some perspective on anaemia treatment in this population.
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Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype
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