Clinical course and outcome after kidney transplantation in patients with C3 glomerulonephritis due to CFHR5 nephropathy.

Autor:	Frangou E; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Cyprus, Nicosia, Cyprus.; Biomedical Research Foundation, Academy of Athens, Athens, Greece., Varnavidou-Nicolaidou A; Histocompatibility and Immunogenetics Laboratory, Nicosia General Hospital, Nicosia, Cyprus., Petousis P; UCLA Bioengineering Department, Los Angeles, CA, USA., Soloukides A; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Cyprus, Nicosia, Cyprus., Theophanous E; Department of Histopathology, Nicosia General Hospital, Nicosia, Cyprus., Savva I; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Molecular Medicine Research Center, Department of Biological Sciences, University of Cyprus, Nicosia, Cyprus., Michael N; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Nicosia, Nicosia, Cyprus., Toumasi E; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Cyprus, Nicosia, Cyprus., Georgiou D; Histocompatibility and Immunogenetics Laboratory, Nicosia General Hospital, Nicosia, Cyprus., Stylianou G; Histocompatibility and Immunogenetics Laboratory, Nicosia General Hospital, Nicosia, Cyprus., Mean R; Histocompatibility and Immunogenetics Laboratory, Nicosia General Hospital, Nicosia, Cyprus., Anastasiadou N; Department of Histopathology, Nicosia General Hospital, Nicosia, Cyprus., Athanasiou Y; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Cyprus, Nicosia, Cyprus., Zavros M; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus., Kyriacou K; Department of Electron Microscopy, Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus., Deltas C; Molecular Medicine Research Center, Department of Biological Sciences, University of Cyprus, Nicosia, Cyprus., Hadjianastassiou V; Department of Nephrology and Transplantation, Nicosia General Hospital, Nicosia, Cyprus.; Medical School, University of Nicosia, Nicosia, Cyprus.
Jazyk:	angličtina
Zdroj:	Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association [Nephrol Dial Transplant] 2019 Oct 01; Vol. 34 (10), pp. 1780-1788.
DOI:	10.1093/ndt/gfz021
Abstrakt:	Background: Complement factor H-related protein 5 (CFHR5) nephropathy is an inherited renal disease characterized by microscopic and synpharyngitic macroscopic haematuria, C3 glomerulonephritis and renal failure. It is caused by an internal duplication of exons 2-3 within the CFHR5 gene resulting in dysregulation of the alternative complement pathway. The clinical characteristics and outcomes of transplanted patients with this rare familial nephropathy remain unknown. Methods: This is a retrospective case series study of 17 kidney transplant patients with the established founder mutation, followed-up over a span of 30 years. Results: The mean (±SD) age of patients at the time of the study and at transplantation was 58.6 ± 9.9 and 46.7 ± 8.8 years, respectively. The 10- and 15-year patient survival rates were 100 and 77.8%, respectively. Proteinuria was present in 33.3% and microscopic haematuria in 58.3% of patients with a functional graft. Serum complement levels were normal in all. 'Confirmed' and 'likely' recurrence of CFHR5 nephropathy were 16.6 and 52.9%, respectively; however, 76.5% of patients had a functional graft after a median of 120 months post-transplantation. Total recurrence was not associated with graft loss (P = 0.171), but was associated with the presence of microscopic haematuria (P = 0.001) and proteinuria (P = 0.018). Graft loss was associated with the presence of proteinuria (P = 0.025). Conclusions: We describe for the first time the clinical characteristics and outcome of patients with CFHR5 nephropathy post-transplantation. Despite the recurrence of CFHR5 nephropathy, we provide evidence for a long-term favourable outcome and support the continued provision of kidney transplantation as a renal replacement option in patients with CFHR5 nephropathy. (© The Author(s) 2019. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.)
Databáze:	MEDLINE
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