Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference

Timothy H J Goodship; H Terence Cook; Fadi Fakhouri; Fernando C Fervenza; Véronique Frémeaux-Bacchi; David Kavanagh; Carla M Nester; Marina Noris; Matthew C Pickering; Santiago Rodriguez de Cordoba; Lubka T Roumenina; Sanjeev Sethi; Richard J H Smith

doi:10.1016/j.kint.2016.10.005

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Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference

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Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference

Timothy H J Goodship, H Terence Cook, Fadi Fakhouri, Fernando C Fervenza, Véronique Frémeaux-Bacchi, David Kavanagh, Carla M Nester, Marina Noris, Matthew C Pickering, Santiago Rodriguez de Cordoba, …

Kidney international, Vol.91(3), pp.539-551

03/2017

DOI: 10.1016/j.kint.2016.10.005

PMID: 27989322

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Abstract

In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.

Phenotype

Genetic Predisposition to Disease

Kidney - drug effects

Kidney - pathology

Humans

Risk Factors

Glomerulonephritis - genetics

Kidney - immunology

Glomerulonephritis - immunology

Treatment Outcome

Atypical Hemolytic Uremic Syndrome - immunology

Complement C3 - immunology

Glomerulonephritis - drug therapy

Atypical Hemolytic Uremic Syndrome - pathology

Animals

Glomerulonephritis - pathology

Complement Inactivating Agents - therapeutic use

Atypical Hemolytic Uremic Syndrome - drug therapy

Atypical Hemolytic Uremic Syndrome - genetics

Complement C3 - genetics

Complement Activation - drug effects

Details

Title: Subtitle: Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference
Creators: Timothy H J Goodship - Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK. Electronic address: tim.goodship@ncl.ac.uk
H Terence Cook - Centre for Complement and Inflammation Research, Department of Medicine, Imperial College Hammersmith Campus, London, UK
Fadi Fakhouri - INSERM, UMR-S 1064, and Department of Nephrology and Immunology, CHU de Nantes, Nantes, France
Fernando C Fervenza - Department of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA
Véronique Frémeaux-Bacchi - Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France
David Kavanagh - Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
Carla M Nester - Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA; Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA
Marina Noris - IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò," Ranica, Bergamo, Italy
Matthew C Pickering - Centre for Complement and Inflammation Research, Department of Medicine, Imperial College Hammersmith Campus, London, UK
Santiago Rodriguez de Cordoba - Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain; Centro de Investigación Biomédica en Enfermedades Raras, Madrid, Spain
Lubka T Roumenina - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche S1138, Complément et Maladies, Centre de Recherche des Cordeliers, Paris, France; Université Paris Descartes Sorbonne Paris-Cité, Paris, France; Université Pierre et Marie Curie (UPMC-Paris-6), Paris, France
Sanjeev Sethi - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
Richard J H Smith - Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA; Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA. Electronic address: richard-smith@uiowa.edu
Resource Type: Journal article
Publication Details: Kidney international, Vol.91(3), pp.539-551
DOI: 10.1016/j.kint.2016.10.005
PMID: 27989322
NLM abbreviation: Kidney Int
ISSN: 0085-2538
eISSN: 1523-1755
Publisher: United States
Grant note: G1002528 / Medical Research Council
Language: English
Date published: 03/2017
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984006464402771

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