Deficiency of CFHR plasma proteins and autoantibody positive hemolytic uremic syndrome: treatment rationale, outcomes, and monitoring.

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Franca Iorember, Anjali Nayak

Abstract

Deficiency of Complement Factor H Related (CFHR) plasma proteins and Autoantibody Positive Hemolytic Uremic Syndrome (DEAP-HUS) is a subtype of atypical hemolytic uremic syndrome, known to be associated with significant morbidity. Its pathogenesis is linked to the production of IgG autoantibodies against complement factor H, a regulator of the alternative complement pathway. The binding of the autoantibodies to the C terminal of complement factor H interferes with its regulatory function, leading to increased activation of the alternative complement pathway and consequent endothelial cellular damage. Early diagnosis and initiation of appropriate therapy is reported to lead to favorable outcomes. Institution of plasma exchange therapy within 24 h of diagnosis has been shown to rapidly lower antibody levels, leading to clinical improvement. Adjunctive immunosuppression therapy suppresses antibody production and helps in maintaining long-term clinical remission of the disease. Available data advocates a treatment regimen that combines plasma therapy (preferably plasma exchange) and immunosuppression to halt disease process and sustain long-term disease remission.

References

Jan 5, 1999·Clinical and Diagnostic Laboratory Immunology·T D JaskowskiH R Hill
Nov 11, 2003·Clinical and Diagnostic Laboratory Immunology·Michael Kirschfink, Tom E Mollnes
Apr 20, 2006·Blood·Jessica CaprioliUNKNOWN International Registry of Recurrent and Familial HUS/TTP
Mar 11, 2008·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Theresa KwonChantal Loirat
Jul 3, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Chantal LoiratVéronique Fremeaux-Bacchi
Sep 18, 2008·Blood·Veronique Frémeaux-BacchiJohn P Atkinson
Mar 27, 2009·Nature Reviews. Nephrology·Marina Noris, Giuseppe Remuzzi
Apr 24, 2009·Vaccine·Christine Skerka, Peter F Zipfel
Jul 21, 2009·Immunology Letters·Peter F Zipfel
Jul 25, 2009·The New England Journal of Medicine·Mieke DelvaeyeEdward M Conway
Aug 12, 2009·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Stefanie StrobelMihály Józsi
Oct 23, 2009·The New England Journal of Medicine·Marina Noris, Giuseppe Remuzzi
Mar 6, 2010·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Olivia BoyerPatrick Niaudet
Mar 31, 2010·Journal of Visualized Experiments : JoVE·Maurizio Costabile
Sep 25, 2010·Seminars in Thrombosis and Hemostasis·Christine SkerkaJohannes Hofer
Sep 25, 2010·Seminars in Thrombosis and Hemostasis·Marie-Agnès Dragon-DureyLothar-Bernd Zimmerhackl
Mar 14, 2012·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Lianne M GeerdinkNicole C A J van de Kar
Jul 9, 2013·Molecular Immunology·Christine SkerkaLubka T Roumenina
Jul 23, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Gwenaëlle SanaOlivia Boyer
Nov 20, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Damien NooneChristoph Licht
May 21, 2014·Pediatrics·Benedetta Diamante ChiodiniBrigitte Adams
Sep 14, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Priyanka KhandelwalArvind Bagga
Apr 11, 2015·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Chantal LoiratUNKNOWN HUS International
May 10, 2015·Immunobiology·B Paul Morgan
Oct 7, 2015·American Journal of Hematology·Angela R Shih, Mandakolathur R Murali
Jun 10, 2016·The Journal of Immunology : Official Journal of the American Association of Immunologists·Denise BuhlmannChristine Skerka
Jul 28, 2016·Nature Reviews. Nephrology·Marie-Agnes Dragon DureyArvind Bagga
Aug 1, 2016·Immunobiology·Zoltán ProhászkaMichael Kirschfink
Feb 22, 2017·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Agnes HacklSandra Habbig
Jun 26, 2017·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Bénédicte Puissant-LubranoAntoine Blancher
Jul 14, 2017·MMWR. Morbidity and Mortality Weekly Report·Lucy A McNamaraJessica R MacNeil
Jan 18, 2018·Journal of the American Society of Nephrology : JASN·Stefan MichelfelderKarsten Häffner
Nov 24, 2019·Seminars in Immunology·Marcell CserhalmiMihály Józsi
Nov 28, 2019·Journal of Immunoassay & Immunochemistry·Kheir Eddine Kerboua, Kamal Djenouhat

❮ 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

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Peter F ZipfelEuropean DEAP-HUS Study Group
Clinical Journal of the American Society of Nephrology : CJASN
David KavanaghKevin J Marchbank
Current Opinion in Rheumatology
Peter F Zipfel, Christine Skerka
Current Opinion in Nephrology and Hypertension
David Kavanagh, Timothy H Goodship
© 2022 Meta ULC. All rights reserved