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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A novel missense mutation in complement factor I predisposes patients to atypical hemolytic uremic syndrome: a case report
TL;DR: In this article , a 26-year-old Asian man presented with pulmonary infection, elevated blood pressure, microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.
Journal ArticleDOI
Acute Kidney Damage: Definition, Classification and Optimal Time of Hemodialysis
Nedim Hamzagic,Tomislav Nikolic,Biljana Popovska Jovicic,Petar Canovic,Sasa Jacovic,Dejan Petrovic +5 more
TL;DR: The causes of acute kidney damage in these patients may be prerenal, renal and postrenal as discussed by the authors, which is the most common cause of the development of acute renal damage in intensive care units.
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The Complement System in the Modern Era of Kidney Transplantation: Mechanisms of Injury and Targeted Therapies.
TL;DR: In this paper , the authors highlight the important role of the complement system at every stage of the kidney transplantation process and highlight potential triggers of complement activation in kidney transplant patients and discuss novel therapeutic agents that can inhibit the complement systems.
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Assessing the Impact of Prophylactic Eculizumab on Renal Graft Survival in Atypical Hemolytic Uremic Syndrome
Emily K. Glover,Kate Smith-Jackson,Vicky Brocklebank,Valerie Wilson,Patrick T. Walsh,Emma Montgomery,Edwin K.S. Wong,Sally Johnson,Michal Malina,David J. Kavanagh,Neil S. Sheerin +10 more
TL;DR: Prophylactic eculizumab treatment at the time of transplantation is used in selected patients with aHUS as mentioned in this paper , which dramatically improves graft survival making transplantation a viable therapeutic option.
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Alternative pathway activation in pregnancy, a measured amount “complements” a successful pregnancy, too much results in adverse events
TL;DR: In this paper , both the physiological role of the alternative pathway of complement in facilitating a normal pregnancy, and its detrimental participation in pregnancy-specific disorders, are discussed, as well as its detrimental role in pregnancy specific disorders.
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