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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Nephrology research—the past, present and future
TL;DR: Five Nature Reviews Nephrology Advisory Board members, who have been associated with the journal since its launch in November 2005, reflect on the progress and roadblocks of the past 10 years and comment on areas where effort and money should be invested and how they expect the field to progress in the next 10 years.
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Potential influences of complement factor H in autoimmune inflammatory and thrombotic disorders
TL;DR: A number of interaction pathways in coagulation and in immunity, together with associated diseases, are reviewed, and where Factor H may be expected to exert an influence is indicated, based on reports of the diversity of ligands for Factor H.
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The formin INF2 in disease: progress from 10 years of research
TL;DR: The structure, biochemical features and functions of INF2 are summarized, a systematic and comprehensive analysis of the pathogenic INF2 mutations are conducted, and a series of conclusions that could be useful for INF2-related disease diagnosis are drawn.
Journal Article
Y402H Polymorphism of Complement Factor H Affects Binding Affinity to C-Reactive Protein
Ilkka Immonen,Matti Laine,Hanna Jarva,Sanna Seitsonen,Karita Haapasalo,Markus J. Lehtinen,Nina Lindeman,Irma Järvelä,S. T. Jokiranta,Seppo Meri +9 more
TL;DR: It is proposed that the reduced binding of FH402H to CRP could lead to an impaired targeting of Fh to cellular debris and a reduction in debris clearance and enhanced inflammation along the macular retinal pigmented epithelium-choroid interface in individuals with age-related macular degeneration.
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Complement activation in thrombotic microangiopathies.
TL;DR: Plasma samples of 81 patients diagnosed with thrombotic microangiopathies were studied according to clinical criteria for functional abnormalities in both ADAMTS-13 and complement regulation and complement activity was measured by the hemolysis of sheep erythrocytes after incubation with human serum or plasma according to modified techniques from Sanchez-Corral, et al.
References
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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.
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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