Eculizumab in Pediatric Dense Deposit Disease

Clinical Journal of the American Society of Nephrology : CJASN
Michiel J S OosterveldJaap W Groothoff

Abstract

Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease affecting mostly children. Treatment options are limited. Debate exists whether eculizumab, a monoclonal antibody against complement factor C5, is effective in DDD. Reported data are scarce, especially in children. The authors analyzed clinical and histologic data of five pediatric patients with a native kidney biopsy diagnosis of DDD. Patients received eculizumab as therapy of last resort for severe nephritic or nephrotic syndrome with alternative pathway complement activation; this therapy was given only when the patients had not or only marginally responded to immunosuppressive therapy. Outcome measures were kidney function, proteinuria, and urine analysis. In all, seven disease episodes were treated with eculizumab (six episodes of severe nephritic syndrome [two of which required dialysis] and one nephrotic syndrome episode). Median age at treatment start was 8.4 (range, 5.9-13) years. For three treatment episodes, eculizumab was the sole immunosuppressive treatment. In all patients, both proteinuria and renal function improved significantly within 12 weeks of treatment (median urinary protein-to-creatinine ratio of 8.5 [range, 2.2-17] versus 1.1...Continue Reading

References

Apr 30, 2002·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Luis CarrerasMarta Carrera
Feb 6, 2004·The New England Journal of Medicine·Peter HillmenRussell P Rother
Jun 14, 2006·Proceedings of the National Academy of Sciences of the United States of America·M C PickeringM Botto
Aug 7, 2007·Journal of the American Society of Nephrology : JASN·Richard J H SmithUNKNOWN Dense Deposit Disease Focus Group
Oct 31, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Samih H NasrVivette D D'Agati
Nov 13, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Bassam Alchi, David Jayne
Jul 8, 2010·Nature Reviews. Nephrology·Fadi FakhouriMatthew C Pickering
Jan 11, 2011·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Joseph R AngeloMichael C Braun
Nov 23, 2011·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Der-Fa LuRichard J H Smith
Jan 12, 2012·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·J A McCaughanA E Courtney
Mar 10, 2012·Clinical Journal of the American Society of Nephrology : CJASN·Andrew S BombackGerald B Appel
Mar 23, 2012·The New England Journal of Medicine·Erica DainaGiuseppe Remuzzi
Mar 23, 2012·The New England Journal of Medicine·Marina VivarelliFrancesco Emma
Jun 9, 2012·Journal of the American Society of Nephrology : JASN·Leal C HerlitzVivette D D'Agati
Aug 1, 2012·Kidney International·Vivette D D'Agati, Andrew S Bomback
Oct 3, 2012·Nature Reviews. Nephrology·Julien ZuberUNKNOWN French Study Group for aHUS/C3G
Nov 13, 2012·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Jan SchmidtkoPascal Meier
Jan 16, 2013·Current Opinion in Nephrology and Hypertension·Carla M Nester, Richard J Smith
May 22, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sevgi GurkanRichard J Smith
Jun 7, 2013·The New England Journal of Medicine·C M LegendreC Loirat
Sep 27, 2013·Kidney International·Jean HouVivette D D'Agati
Nov 1, 2013·Kidney International·Matthew C PickeringH Terence Cook
Jan 11, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Caroline Rousset-RouvièreMichel Tsimaratos
Jan 28, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Ozan OzkayaHasan Tahsin Keceligil
Jun 9, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Ana Sánchez-MorenoPatricia Ybot-González

❮ Previous
Next ❯

Citations

Jan 14, 2016·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Cheryl L TranFernando C Fervenza
Apr 7, 2016·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Rebecca C Graves, Richard N Fine
May 9, 2016·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Lindsay S Keir, Craig B Langman
May 11, 2016·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Chia Wei TeohChristoph Licht
Jul 13, 2016·Seminars in Immunology·Carla M Nester, Richard J H Smith
Aug 17, 2016·Clinical Journal of the American Society of Nephrology : CJASN·Shveta S MotwaniUNKNOWN American Society of Nephrology Onco-Nephrology Forum
Nov 24, 2016·Clinical and Experimental Nephrology·Naoko ItoMichio Nagata
Jan 14, 2018·BMC Nephrology·Thomas WelteElke Neumann-Haefelin
Mar 14, 2019·Current Opinion in Nephrology and Hypertension·Ramy M HannaRichard Burwick
Mar 11, 2017·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Farah A FalixAntonia H M Bouts
Jan 5, 2019·Nature Reviews. Nephrology·Noemie Jourde-ChicheLubka T Roumenina
Sep 22, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Johannes HolleJulia Thumfart
Oct 25, 2020·Medical Sciences : Open Access Journal·Maria L Gonzalez SuarezWisit Cheungpasitporn
Nov 3, 2020·Molecular Immunology·Felix Poppelaars, Joshua M Thurman
Mar 12, 2021·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Melissa Muff-LuettCarla M Nester
May 19, 2021·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marina VivarelliJoshua M Thurman
Feb 1, 2017·Kidney International·Allison Lesher WilliamsWen-Chao Song
Sep 4, 2021·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marloes A H M MichelsNicole C A J van de Kar

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Case Reports in Nephrology
Aurore Berthe-AucejoGeorges Deschênes
Clinical Journal of the American Society of Nephrology : CJASN
Debbie S GipsonMatthias Kretzler
Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Caroline Rousset-RouvièreMichel Tsimaratos
Clinical Journal of the American Society of Nephrology : CJASN
Andrew S BombackGerald B Appel
© 2021 Meta ULC. All rights reserved