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
Complement activation patterns in atypical haemolytic uraemic syndrome during acute phase and in remission
Elena B. Volokhina,Dineke Westra,T.J.A.M. Van der Velden,N.C.A.J. van de Kar,Tom Eirik Mollnes,L.P.W.J. van den Heuvel,L.P.W.J. van den Heuvel +6 more
TL;DR: Serum samples from patients in remission showed normal in vitro patterns of complement activation and demonstrated normal kinetics of complementactivation in the fluid phase, providing important insight into complement regulation in aHUS.
Journal ArticleDOI
A De Novo Deletion in the Regulators of Complement Activation Cluster Producing a Hybrid Complement Factor H/Complement Factor H–Related 3 Gene in Atypical Hemolytic Uremic Syndrome
Rachel C. Challis,Geisilaine S.R. Araujo,Edwin K.S. Wong,Holly E. Anderson,Atif Awan,Anthony Dorman,Mary Waldron,Valerie Wilson,Vicky Brocklebank,Lisa Strain,B. Paul Morgan,Claire L. Harris,Kevin J. Marchbank,Timothy H.J. Goodship,David J. Kavanagh +14 more
TL;DR: The formation of this novel CFH/CFHR3 hybrid gene secondary to a de novo 6.3-kb deletion that arose through microhomology-mediated end joining rather than nonallelic homologous recombination suggests that this cluster is a dynamic area of the genome in which additional genomic disorders may arise.
Journal ArticleDOI
Thrombotic Microangiopathy in Inverted Formin 2–Mediated Renal Disease
Rachel C. Challis,Troels Ring,Yaobo Xu,Edwin K.S. Wong,Oliver Flossmann,Ian S.D. Roberts,Saeed Ahmed,Michael Wetherall,Giedrius Salkus,Vicky Brocklebank,Julian Fester,Lisa Strain,Valerie Wilson,Katrina M Wood,Kevin J. Marchbank,Mauro Santibanez-Koref,Timothy H.J. Goodship,David J. Kavanagh +17 more
TL;DR: Identifying individuals with TMAs who may not respond to eculizumab will avoid prolonged exposure of such individuals to the infectious complications of terminal pathway C blockade, and potentially accounting for the genetic pleiotropy.
Journal ArticleDOI
The genetics of atypical hemolytic uremic syndrome.
Wouter J C Feitz,Nicole C. A. J. van de Kar,Dorothea Orth-Höller,Lambert P J W van den Heuvel,Lambert P J W van den Heuvel,Christoph Licht +5 more
TL;DR: The genetic background of aH US, the role of complement for aHUS pathogenesis, and the different groups of specific mutations known to be involved in the pathogenesis are discussed.
Journal ArticleDOI
Pediatric Antiphospholipid Syndrome
TL;DR: The purpose of this report is to review the current literature on APS in children and neonates, identify the gaps in current knowledge, and suggest avenues for studies to fill those gaps.
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