C3 glomerulopathy and atypical hemolytic uremic syndrome: an updated review of the literature on alternative complement pathway disorders

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dc.contributor.authorTurkmen, Kultigin
dc.contributor.authorBaloglu, Ismail
dc.contributor.authorOzer, Hakan
dc.date.accessioned2024-02-23T13:59:28Z
dc.date.available2024-02-23T13:59:28Z
dc.date.issued2021
dc.departmentNEÜen_US
dc.description.abstractThe complement system plays a significant role within the pathological process of C3 glomerulopathy (C3GP) and atypical hemolytic uremic syndrome (aHUS). In daily practice, clinicians should differentiate the subgroups of C3GP because of they should apply different treatment modalities. In the past, C3GP was considered as a part of membranoproliferative glomerulonephritis (MPGN). MPGN is defined as glomerular capillary thickening secondary to the synthesis of the new glomerular basement membrane and mesangial cellular hyperplasia with mesangial matrix expansion. Atypical hemolytic uremic syndrome is an ultra-rare disease that can be outlined by the triad of Coombs negative microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Recent advances demonstrated that these diseases share common abnormalities of the control of the alternative complement system. Therefore, nowadays, most researchers advocate that there may be overlap in the pathogenesis of C3GP and aHUS. This review will provide recent novel mechanisms and treatment options in these diseases. For the purposes that we mentioned above and to help clinicians, we aimed to describe the etiology, pathophysiology, and treatment of C3GP and aHUS in this comprehensive review.en_US
dc.description.sponsorshipAlexion Pharma Turkeyen_US
dc.description.sponsorshipThe authors thank for the assistance in English editing, provided by Anna H Moosuddee of Global Editing Service, UK which was funded by Alexion Pharma Turkey.en_US
dc.identifier.doi10.1007/s11255-020-02729-y
dc.identifier.endpage2080en_US
dc.identifier.issn0301-1623
dc.identifier.issn1573-2584
dc.identifier.issue10en_US
dc.identifier.pmid33389509en_US
dc.identifier.scopus2-s2.0-85098574804en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage2067en_US
dc.identifier.urihttps://doi.org/10.1007/s11255-020-02729-y
dc.identifier.urihttps://hdl.handle.net/20.500.12452/11209
dc.identifier.volume53en_US
dc.identifier.wosWOS:000604575300021en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofInternational Urology And Nephrologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAtypical Hemolytic Uremic Syndromeen_US
dc.subjectComplement 3 Glomerulopathyen_US
dc.subjectEculizumaben_US
dc.subjectAvacopanen_US
dc.titleC3 glomerulopathy and atypical hemolytic uremic syndrome: an updated review of the literature on alternative complement pathway disordersen_US
dc.typeReview Articleen_US

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