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
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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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