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
Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation
Sejin Kim,Eujin Park,Eujin Park,Sang Il Min,Nam-Joon Yi,Jongwon Ha,Il-Soo Ha,Hae Il Cheong,Hae Il Cheong,Hee Gyung Kang,Hee Gyung Kang +10 more
TL;DR: In patients with aH US associated with a CFH mutation, KT without LT was complicated with a recurrence of aHUS, which might lead to allograft loss, and LT was successful in preventing the recurrence in patients with defective CFH.
Journal ArticleDOI
The causes and consequences of paediatric kidney disease on adult nephrology care.
Ruth J. Pepper,RS Trompeter +1 more
TL;DR: In this article, a review discusses common causes of childhood chronic kidney disease, in particular congenital anomalies of the kidney and urinary tract (CAKUT), autosomal dominant tubulointerstitial kidney disease (ADTKD), polycystic kidney diseases, hereditary stone disease, nephrotic syndrome and atypical haemolytic uraemic syndrome.
Journal ArticleDOI
How I Treat Complement-Mediated TMA
TL;DR: In this paper , a 37-year-old woman with ulcerative colitis on 6-mercaptopurine presented to the hospital with 2 weeks of intermittent vomiting and BP of 152/100 mm Hg.
Journal ArticleDOI
Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern
Camino García Monteavaro,Carmen Peralta Roselló,Borja Quiroga,José María Baltar Martín,Lorena Castillo Eraso,Fernando de Álvaro Moreno,Alberto Martínez Vea,María Teresa Visus-Fernández de Manzanos +7 more
TL;DR: Three patients with atypical hemolytic uremic syndrome and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizer treatment protocol are presented.
Journal ArticleDOI
Atypical hemolytic uremic syndrome and eculizumab therapy in children.
TL;DR: Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS and is recommended as the first-line therapy in children with aH US.
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