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
Genotype-phenotype correlations of low-frequency variants in the complement system in renal disease and age-related macular degeneration.
Maartje J. Geerlings,Elena B. Volokhina,Elena B. Volokhina,E.K. de Jong,N.C.A.J. van de Kar,Marc Pauper,Carel B. Hoyng,L.P.W.J. van den Heuvel,L.P.W.J. van den Heuvel,L.P.W.J. van den Heuvel,A.I. den Hollander +10 more
TL;DR: A substantial overlap of variants is observed between aHUS/C3G and AMD; however, there is a distinct clustering of variants within specific domains.
Journal ArticleDOI
Complement and glomerular diseases.
Reena J Popat,Michael G. Robson +1 more
TL;DR: An overview of the role of complement in glomerular diseases is provided and the data from patients and animal models with reference to specific diseases are discussed.
Journal ArticleDOI
Membrane cofactor protein (MCP; CD46): deficiency states and pathogen connections.
TL;DR: In this paper, a review focuses on recent advances in identifying disease-causing CD46 variants and its pathogen connections. But the authors do not consider the role of CD46 in human reproduction, autophagy, modulating T cell activation and effector functions.
Journal ArticleDOI
Postoperative atypical hemolytic uremic syndrome associated with complement c3 mutation.
Eiji Matsukuma,Atsushi Imamura,Yusuke Iwata,Takamasa Takeuchi,Yoko Yoshida,Yoshihiro Fujimura,Xinping Fan,Toshiyuki Miyata,Takashi Kuwahara +8 more
TL;DR: A case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation is reported, which is a potentially causative mutation in complement component C3.
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Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab.
Gabriela Andries,Michael Karass,Srikanth Yandrapalli,Katherine Linder,Delong Liu,John C. Nelson,Rahul Pawar,Savneek Chugh +7 more
TL;DR: This case suggests that treatment of atypical hemolytic uremic syndrome in early trimester of pregnancy with eculizumab results in good outcome to mother and fetus.
References
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Journal ArticleDOI
Complement: a key system for immune surveillance and homeostasis
TL;DR: An updated view of the function, structure and dynamics of the complement network is described, its interconnection with immunity at large and with other endogenous pathways is highlighted, and its multiple roles in homeostasis and disease are illustrated.
Journal ArticleDOI
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
TL;DR: Eculizumab inhibited complement-mediated thrombotic microangiopathy and was associated with significant time-dependent improvement in renal function in patients with atypical hemolytic-uremic syndrome and was also associated with improvement in health-related quality of life.
Journal ArticleDOI
Atypical Hemolytic–Uremic Syndrome
Marina Noris,Giuseppe Remuzzi +1 more
TL;DR: Current concepts about the pathobiology of atypical hemolytic–uremic syndrome are reviewed and its diagnosis and management are reviewed.
Journal ArticleDOI
Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype
Marina Noris,Jessica Caprioli,Elena Bresin,Chiara Mossali,Gaia Pianetti,Sara Gamba,Erica Daina,Chiara Fenili,Federica Castelletti,Annalisa Sorosina,Rossella Piras,Roberta Donadelli,Ramona Maranta,Irene van der Meer,Edward M. Conway,Peter F. Zipfel,Timothy H.J. Goodship,Giuseppe Remuzzi +17 more
TL;DR: Results underline the need of genetic screening for all susceptibility factors as part of clinical management of aHUS and for identification of patients who could safely benefit from kidney transplant.
Journal ArticleDOI
Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria
TL;DR: The recent approval of eculizumab as a first-in-class complement inhibitor for the treatment of PNH validates the concept of complement inhibition as an effective therapy and provides rationale for investigation of other indications in which complement plays a role.
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