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

被引：503

作者：

Goodship, Timothy H. J. ^{[1
]}

Cook, H. Terence ^{[2
]}

Fakhouri, Fadi ^{[3
,4
]}

Fervenza, Fernando C. ^{[5
]}

Fremeaux-Bacchi, Veronique ^{[6
]}

Kavanagh, David ^{[1
]}

Nester, Carla M. ^{[7
,8
]}

Noris, Marina ^{[9
]}

Pickering, Matthew C. ^{[2
]}

de Cordoba, Santiago Rodriguez ^{[10
,11
]}

Roumenina, Lubka T. ^{[12
,13
,14
]}

Sethi, Sanjeev ^{[15
]}

Smith, Richard J. H. ^{[7
,8
]}

机构：

[1] Newcastle Univ, Inst Med Genet, Newcastle Upon Tyne NE1 3BZ, Tyne & Wear, England

[2] Ctr Complement & Inflammat Res, Dept Med, Imperial Coll Hammersmith Campus, London, England

[3] CHU Nantes, INSERM, UMR S 1064, Nantes, France

[4] CHU Nantes, Dept Nephrol & Immunol, Nantes, France

[5] Mayo Clin, Dept Hypertens & Nephrol, Rochester, MN USA

[6] Hop Europeen Georges Pompidou, AP HP, Paris, France

[7] Univ Iowa, Carver Coll Med, Mol Otolaryngol & Renal Res Labs, Iowa City, IA USA

[8] Univ Iowa, Carver Coll Med, Dept Internal Med, Div Nephrol, Iowa City, IA USA

[9] IRCCS, Ist Ric Farmacol Mario Negri, Clin Res Ctr Rare Dis Aldo & Cele Dacco Ranica, Bergamo, Italy

[10] CSIC, Ctr Invest Biol, Madrid, Spain

[11] Ctr Invest Biomed Enfermedades Raras, Madrid, Spain

[12] Inst Natl Sante & Rech Med, Ctr Rech Cordeliers, Unite Mixte Rech 51138, Complement & Malad, Paris, France

[13] Univ Paris 05, Sorbonne Paris Cite, Paris, France

[14] UPMC Paris 6, Paris, France

[15] Mayo Clin, Dept Lab Med & Pathol, Rochester, MN USA

来源：

KIDNEY INTERNATIONAL | 2017年 / 91卷 / 03期

基金：

英国惠康基金; 英国医学研究理事会;

关键词：

anti-complement therapies; atypical hemolytic uremic syndrome; C3; glomerulopathy; complement; glomerulonephritis; kidney disease; COMPLEMENT FACTOR-H; DENSE-DEPOSIT DISEASE; ECULIZUMAB MAINTENANCE TREATMENT; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; MESANGIOCAPILLARY GLOMERULONEPHRITIS; RENAL-TRANSPLANTATION; OXFORD CLASSIFICATION; MACULAR DEGENERATION; ALTERNATIVE PATHWAY; CLINICAL-FEATURES;

D O I：

10.1016/j.kint.2016.10.005

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：539 / 551

页数：13

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