Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant

Abstract Background Rare diseases (RDs) encompass many difficult-to-treat conditions with different characteristics often associated with end-stage renal disease (ESRD). However, data about transplant outcomes in adult patients are still lacking and limited to case reports/case series without differ...

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Autores principales:	Ester Gallo, Silvia Mingozzi, Alberto Mella, Fabrizio Fop, Roberto Presta, Manuel Burdese, Elena Boaglio, Maria Cristina Torazza, Roberta Giraudi, Gianluca Leonardi, Antonio Lavacca, Paolo Gontero, Omidreza Sedigh, Andrea Bosio, Aldo Verri, Caterina Dolla, Luigi Biancone
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Rare diseases Kidney transplantation Genetic renal diseases Survival Primary glomerulonephritis Diseases of the genitourinary system. Urology RC870-923
Acceso en línea:	https://doaj.org/article/c3bbfb4cb57d46f99b4c78679d9ae1b9
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spelling	oai:doaj.org-article:c3bbfb4cb57d46f99b4c78679d9ae1b92021-11-21T12:42:31ZClinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant10.1186/s12882-021-02571-z1471-2369https://doaj.org/article/c3bbfb4cb57d46f99b4c78679d9ae1b92021-11-01T00:00:00Zhttps://doi.org/10.1186/s12882-021-02571-zhttps://doaj.org/toc/1471-2369Abstract Background Rare diseases (RDs) encompass many difficult-to-treat conditions with different characteristics often associated with end-stage renal disease (ESRD). However, data about transplant outcomes in adult patients are still lacking and limited to case reports/case series without differentiation between immunological/non-immunological RDs. Methods Retrospective analysis among all adult kidney transplanted patients (KTs) with RDs (RDsKT group) performed in our high-volume transplantation center between 2005 and 2016. RDs were classified according to the Orphanet code system differentiating between immunological and non-immunological diseases, also comparing clinical outcomes and temporal trends to a control population without RDs (nRDsKT). Results Among 1381 KTs, 350 patients (25.3%) were affected by RDs (RDsKTs). During a f/up > 5 years [median 7.9 years (4.8–11.1)], kidney function and graft/patient survival did not differ from nRDsKTs. Considering all post-transplant complications, RDsKTs (including, by definition, patients with primary glomerulopathy except on IgA nephropathy) have more recurrent and de-novo glomerulonephritis (14.6% vs. 9.6% in nRDsKTs; p = 0.05), similar rates of de-novo cancers, post-transplant diabetes, dysmetabolism, hematologic disorders, urologic/vascular problems, and lower infectious episodes than nRDsKTs (63.7% vs 72.7%; p = 0.013). Additional stratification for immunological and non-immunological RDsKTs or transplantation periods (before/after 2010) showed no differences or temporal trends between groups. Conclusions Kidney transplant centers are deeply involved in RDs management. Despite their high-complex profile, both immunological and non-immunological RDsKTs experienced favorable patients’ and graft survival.Ester GalloSilvia MingozziAlberto MellaFabrizio FopRoberto PrestaManuel BurdeseElena BoaglioMaria Cristina TorazzaRoberta GiraudiGianluca LeonardiAntonio LavaccaPaolo GonteroOmidreza SedighAndrea BosioAldo VerriCaterina DollaLuigi BianconeBMCarticleRare diseasesKidney transplantationGenetic renal diseasesSurvivalPrimary glomerulonephritisDiseases of the genitourinary system. UrologyRC870-923ENBMC Nephrology, Vol 22, Iss 1, Pp 1-13 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Rare diseases Kidney transplantation Genetic renal diseases Survival Primary glomerulonephritis Diseases of the genitourinary system. Urology RC870-923
spellingShingle	Rare diseases Kidney transplantation Genetic renal diseases Survival Primary glomerulonephritis Diseases of the genitourinary system. Urology RC870-923 Ester Gallo Silvia Mingozzi Alberto Mella Fabrizio Fop Roberto Presta Manuel Burdese Elena Boaglio Maria Cristina Torazza Roberta Giraudi Gianluca Leonardi Antonio Lavacca Paolo Gontero Omidreza Sedigh Andrea Bosio Aldo Verri Caterina Dolla Luigi Biancone Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
description	Abstract Background Rare diseases (RDs) encompass many difficult-to-treat conditions with different characteristics often associated with end-stage renal disease (ESRD). However, data about transplant outcomes in adult patients are still lacking and limited to case reports/case series without differentiation between immunological/non-immunological RDs. Methods Retrospective analysis among all adult kidney transplanted patients (KTs) with RDs (RDsKT group) performed in our high-volume transplantation center between 2005 and 2016. RDs were classified according to the Orphanet code system differentiating between immunological and non-immunological diseases, also comparing clinical outcomes and temporal trends to a control population without RDs (nRDsKT). Results Among 1381 KTs, 350 patients (25.3%) were affected by RDs (RDsKTs). During a f/up > 5 years [median 7.9 years (4.8–11.1)], kidney function and graft/patient survival did not differ from nRDsKTs. Considering all post-transplant complications, RDsKTs (including, by definition, patients with primary glomerulopathy except on IgA nephropathy) have more recurrent and de-novo glomerulonephritis (14.6% vs. 9.6% in nRDsKTs; p = 0.05), similar rates of de-novo cancers, post-transplant diabetes, dysmetabolism, hematologic disorders, urologic/vascular problems, and lower infectious episodes than nRDsKTs (63.7% vs 72.7%; p = 0.013). Additional stratification for immunological and non-immunological RDsKTs or transplantation periods (before/after 2010) showed no differences or temporal trends between groups. Conclusions Kidney transplant centers are deeply involved in RDs management. Despite their high-complex profile, both immunological and non-immunological RDsKTs experienced favorable patients’ and graft survival.
format	article
author	Ester Gallo Silvia Mingozzi Alberto Mella Fabrizio Fop Roberto Presta Manuel Burdese Elena Boaglio Maria Cristina Torazza Roberta Giraudi Gianluca Leonardi Antonio Lavacca Paolo Gontero Omidreza Sedigh Andrea Bosio Aldo Verri Caterina Dolla Luigi Biancone
author_facet	Ester Gallo Silvia Mingozzi Alberto Mella Fabrizio Fop Roberto Presta Manuel Burdese Elena Boaglio Maria Cristina Torazza Roberta Giraudi Gianluca Leonardi Antonio Lavacca Paolo Gontero Omidreza Sedigh Andrea Bosio Aldo Verri Caterina Dolla Luigi Biancone
author_sort	Ester Gallo
title	Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
title_short	Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
title_full	Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
title_fullStr	Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
title_full_unstemmed	Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
title_sort	clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant
publisher	BMC
publishDate	2021
url	https://doaj.org/article/c3bbfb4cb57d46f99b4c78679d9ae1b9
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Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant

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