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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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) |
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DOAJ |
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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 |
work_keys_str_mv |
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