Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.

<h4>Background</h4>Atypical Hemolytic Uremic Syndrome (aHUS) is an ultra-rare disease that potentially leads to kidney graft failure due to ongoing Thrombotic Microangiopathy (TMA). The aim was evaluating the frequency of TMA after kidney transplantation in patients with aHUS in a Brazil...

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Autores principales:	Hong Si Nga, Lilian Monteiro Pereira Palma, Miguel Ernandes Neto, Ida Maria Maximina Fernandes-Charpiot, Valter Duro Garcia, Roger Kist, Silvana Maria Carvalho Miranda, Pedro Augusto Macedo de Souza, Gerson Marques Pereira, Luis Gustavo Modelli de Andrade
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/3f7af28362e7407cbd968ca0db0ec9cf
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spelling	oai:doaj.org-article:3f7af28362e7407cbd968ca0db0ec9cf2021-12-02T20:06:00ZThrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.1932-620310.1371/journal.pone.0258319https://doaj.org/article/3f7af28362e7407cbd968ca0db0ec9cf2021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0258319https://doaj.org/toc/1932-6203<h4>Background</h4>Atypical Hemolytic Uremic Syndrome (aHUS) is an ultra-rare disease that potentially leads to kidney graft failure due to ongoing Thrombotic Microangiopathy (TMA). The aim was evaluating the frequency of TMA after kidney transplantation in patients with aHUS in a Brazilian cohort stratified by the use of the specific complement-inhibitor eculizumab.<h4>Methods</h4>This was a multicenter retrospective cohort study including kidney transplant patients diagnosed with aHUS. We collected data from 118 transplant centers in Brazil concerning aHUS transplanted patients between 01/01/2007 and 12/31/2019. Patients were stratified into three groups: no use of eculizumab (No Eculizumab Group), use of eculizumab for treatment of after transplantation TMA (Therapeutic Group), and use of eculizumab for prophylaxis of aHUS recurrence (Prophylactic Group).<h4>Results</h4>Thirty-eight patients with aHUS who received kidney transplantation were enrolled in the study. Patients' mean age was 30 years (24-40), and the majority of participants was women (63% of cases). In the No Eculizumab Group (n = 11), there was a 91% graft loss due to the TMA. The hazard ratio of TMA graft loss was 0.07 [0.01-0.55], p = 0.012 in the eculizumab Prophylactic Group and 0.04 [0.00-0.28], p = 0.002 in the eculizumab Therapeutic Group.<h4>Conclusion</h4>The TMA graft loss in the absence of a specific complement-inhibitor was higher among the Brazilian cohort of kidney transplant patients. This finding reinforces the need of eculizumab use for treatment of aHUS kidney transplant patients. Cost optimization analysis and the early access to C5 inhibitors are suggested, especially in low-medium income countries.Hong Si NgaLilian Monteiro Pereira PalmaMiguel Ernandes NetoIda Maria Maximina Fernandes-CharpiotValter Duro GarciaRoger KistSilvana Maria Carvalho MirandaPedro Augusto Macedo de SouzaGerson Marques PereiraLuis Gustavo Modelli de AndradePublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 11, p e0258319 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Hong Si Nga Lilian Monteiro Pereira Palma Miguel Ernandes Neto Ida Maria Maximina Fernandes-Charpiot Valter Duro Garcia Roger Kist Silvana Maria Carvalho Miranda Pedro Augusto Macedo de Souza Gerson Marques Pereira Luis Gustavo Modelli de Andrade Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
description	<h4>Background</h4>Atypical Hemolytic Uremic Syndrome (aHUS) is an ultra-rare disease that potentially leads to kidney graft failure due to ongoing Thrombotic Microangiopathy (TMA). The aim was evaluating the frequency of TMA after kidney transplantation in patients with aHUS in a Brazilian cohort stratified by the use of the specific complement-inhibitor eculizumab.<h4>Methods</h4>This was a multicenter retrospective cohort study including kidney transplant patients diagnosed with aHUS. We collected data from 118 transplant centers in Brazil concerning aHUS transplanted patients between 01/01/2007 and 12/31/2019. Patients were stratified into three groups: no use of eculizumab (No Eculizumab Group), use of eculizumab for treatment of after transplantation TMA (Therapeutic Group), and use of eculizumab for prophylaxis of aHUS recurrence (Prophylactic Group).<h4>Results</h4>Thirty-eight patients with aHUS who received kidney transplantation were enrolled in the study. Patients' mean age was 30 years (24-40), and the majority of participants was women (63% of cases). In the No Eculizumab Group (n = 11), there was a 91% graft loss due to the TMA. The hazard ratio of TMA graft loss was 0.07 [0.01-0.55], p = 0.012 in the eculizumab Prophylactic Group and 0.04 [0.00-0.28], p = 0.002 in the eculizumab Therapeutic Group.<h4>Conclusion</h4>The TMA graft loss in the absence of a specific complement-inhibitor was higher among the Brazilian cohort of kidney transplant patients. This finding reinforces the need of eculizumab use for treatment of aHUS kidney transplant patients. Cost optimization analysis and the early access to C5 inhibitors are suggested, especially in low-medium income countries.
format	article
author	Hong Si Nga Lilian Monteiro Pereira Palma Miguel Ernandes Neto Ida Maria Maximina Fernandes-Charpiot Valter Duro Garcia Roger Kist Silvana Maria Carvalho Miranda Pedro Augusto Macedo de Souza Gerson Marques Pereira Luis Gustavo Modelli de Andrade
author_facet	Hong Si Nga Lilian Monteiro Pereira Palma Miguel Ernandes Neto Ida Maria Maximina Fernandes-Charpiot Valter Duro Garcia Roger Kist Silvana Maria Carvalho Miranda Pedro Augusto Macedo de Souza Gerson Marques Pereira Luis Gustavo Modelli de Andrade
author_sort	Hong Si Nga
title	Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
title_short	Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
title_full	Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
title_fullStr	Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
title_full_unstemmed	Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.
title_sort	thrombotic microangiopathy after kidney transplantation: analysis of the brazilian atypical hemolytic uremic syndrome cohort.
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/3f7af28362e7407cbd968ca0db0ec9cf
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Thrombotic microangiopathy after kidney transplantation: Analysis of the Brazilian Atypical Hemolytic Uremic Syndrome cohort.

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