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
The Development of Atypical Hemolytic Uremic Syndrome Depends on Complement C5
Elena Goicoechea de Jorge,Paolo Macor,Danielle Paixão-Cavalcante,Kirsten L. Rose,Franco Tedesco,H. Terence Cook,Marina Botto,Matthew C. Pickering +7 more
TL;DR: A critical role for C5 activation is demonstrated in both spontaneous aHUS and experimentally triggered renal injury in animals with defective complement factor H function, providing a rationale to investigate therapeutic inhibition of C5 in human aH US.
Journal ArticleDOI
Complement Factor H–Related Protein 1 Deficiency and Factor H Antibodies in Pediatric Patients with Atypical Hemolytic Uremic Syndrome
Johannes Hofer,Andreas R. Janecke,Lothar-Bernd Zimmerhackl,Magdalena Riedl,Alejandra Rosales,Thomas Giner,Gerard Cortina,Carola J. Haindl,Barbara Petzelberger,Miriam Pawlik,Verena Jeller,Udo Vester,Bettina Gadner,Michael van Husen,Michael L. Moritz,Reinhard Würzner,Therese Jungraithmayr +16 more
TL;DR: Homozygous deletion in CFHR1 is strongly associated with occurrence of CFH antibodies in pediatric patients with atypical hemolytic uremic syndrome, but it cannot be considered an exclusive cause for aHUS.
Journal ArticleDOI
Overall Neutralization of Complement Factor H by Autoantibodies in the Acute Phase of the Autoimmune Form of Atypical Hemolytic Uremic Syndrome
C. Blanc,Lubka T. Roumenina,Yahya Ashraf,Satu Hyvärinen,Sidharth Kumar Sethi,Bruno Ranchin,Patrick Niaudet,Chantal Loirat,Ashima Gulati,Arvind Bagga,Wolf H. Fridman,Wolf H. Fridman,Catherine Sautès-Fridman,Catherine Sautès-Fridman,T. Sakari Jokiranta,Véronique Frémeaux-Bacchi,Marie-Agnès Dragon-Durey,Marie-Agnès Dragon-Durey +17 more
TL;DR: It is shown that anti-FH autoantibodies induce neutralization of FH at acute phase of the disease, leading to an overall impairment of several functions of Fh, extending the role of autoant ibodies beyond the impairment of the direct cell surface protection.
Journal ArticleDOI
Mutations of Factor H Impair Regulation of Surface-bound C3b by Three Mechanisms in Atypical Hemolytic Uremic Syndrome
Markus J. Lehtinen,Angelique L.W.M.M. Rops,David E. Isenman,Johan van der Vlag,T. Sakari Jokiranta +4 more
TL;DR: It is concluded that binding of FH19–20 to C3b/C3d is essential for target discrimination by the alternative pathway.
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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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