Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience

William Morello, Sairaj Puvinathan, Giuseppe Puccio, Gian Marco Ghiggeri, Luca Dello Strologo, Licia Peruzzi, Luisa Murer, Michela Cioni, Isabella Guzzo, Enrico Cocchi, Elisa Benetti, Sara Testa, Luciana Ghio, Gianluca Caridi, Massimo Cardillo, Rosanna Torelli, Giovanni Montini

Research output: Contribution to journalArticle

Abstract

Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.

Original languageEnglish
JournalJournal of Nephrology
DOIs
Publication statusAccepted/In press - Jan 1 2019

Fingerprint

Nephrotic Syndrome
Steroids
Transplants
Recurrence
Dialysis
Kidney
Mutation
Graft Survival
Age of Onset
Kidney Transplantation
Italy
Chronic Kidney Failure
Histology
Transplantation

Keywords

  • Kidney transplant
  • Post-transplant recurrence
  • Steroid-resistant nephrotic syndrome

ASJC Scopus subject areas

  • Nephrology

Cite this

Post-transplant recurrence of steroid resistant nephrotic syndrome in children : the Italian experience. / Morello, William; Puvinathan, Sairaj; Puccio, Giuseppe; Ghiggeri, Gian Marco; Dello Strologo, Luca; Peruzzi, Licia; Murer, Luisa; Cioni, Michela; Guzzo, Isabella; Cocchi, Enrico; Benetti, Elisa; Testa, Sara; Ghio, Luciana; Caridi, Gianluca; Cardillo, Massimo; Torelli, Rosanna; Montini, Giovanni.

In: Journal of Nephrology, 01.01.2019.

Research output: Contribution to journalArticle

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title = "Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience",
abstract = "Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3{\%} of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24{\%} of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55{\%}) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.",
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author = "William Morello and Sairaj Puvinathan and Giuseppe Puccio and Ghiggeri, {Gian Marco} and {Dello Strologo}, Luca and Licia Peruzzi and Luisa Murer and Michela Cioni and Isabella Guzzo and Enrico Cocchi and Elisa Benetti and Sara Testa and Luciana Ghio and Gianluca Caridi and Massimo Cardillo and Rosanna Torelli and Giovanni Montini",
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T1 - Post-transplant recurrence of steroid resistant nephrotic syndrome in children

T2 - the Italian experience

AU - Morello, William

AU - Puvinathan, Sairaj

AU - Puccio, Giuseppe

AU - Ghiggeri, Gian Marco

AU - Dello Strologo, Luca

AU - Peruzzi, Licia

AU - Murer, Luisa

AU - Cioni, Michela

AU - Guzzo, Isabella

AU - Cocchi, Enrico

AU - Benetti, Elisa

AU - Testa, Sara

AU - Ghio, Luciana

AU - Caridi, Gianluca

AU - Cardillo, Massimo

AU - Torelli, Rosanna

AU - Montini, Giovanni

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.

AB - Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.

KW - Kidney transplant

KW - Post-transplant recurrence

KW - Steroid-resistant nephrotic syndrome

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SN - 1121-8428

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