Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment

BackgroundEarly onset de novo focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized trea...

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Main Authors: Karla Carvajal Abreu, Sebastian Loos, Lutz Fischer, Lars Pape, Thorsten Wiech, Markus J. Kemper, Burkhard Tönshoff, Jun Oh, Raphael Schild
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-09-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fped.2023.1280521/full
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author Karla Carvajal Abreu
Sebastian Loos
Lutz Fischer
Lars Pape
Thorsten Wiech
Markus J. Kemper
Burkhard Tönshoff
Jun Oh
Raphael Schild
author_facet Karla Carvajal Abreu
Sebastian Loos
Lutz Fischer
Lars Pape
Thorsten Wiech
Markus J. Kemper
Burkhard Tönshoff
Jun Oh
Raphael Schild
author_sort Karla Carvajal Abreu
collection DOAJ
description BackgroundEarly onset de novo focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized treatment protocols have not yet been established.Case descriptionWe describe a boy with early onset de novo FSGS in the transplanted kidney and non-selective glomerular proteinuria (maximum albumin-to-creatinine ratio of 3.8 g/g; normal range, ≤0.03 g/g creatinine). Manifestation occurred at 30 days posttransplant and was accompanied by a significant graft dysfunction (eGFR 61 ml/min per 1.73 m2). Treatment with 25 sessions of plasmapheresis over 14 weeks and three consecutive days of methylprednisolone pulse therapy (10 mg/kg per day) followed by oral prednisolone as rejection prophylaxis (3.73 mg/m2 per day) led to sustained remission of proteinuria (albumin-to-creatinine ratio of 0.028 g/g) and normalization of graft function (eGFR 92 ml/min per 1.73 m2) after 14 weeks. The follow-up period was 36 months.ConclusionsThis case underlines the efficacy of immunosuppressive and antibody eliminating therapy in early onset de novo FSGS after kidney transplantation.
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spelling doaj.art-859ec1c8e80e4092951a0bffb6ba70ab2023-09-27T05:18:15ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-09-011110.3389/fped.2023.12805211280521Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatmentKarla Carvajal Abreu0Sebastian Loos1Lutz Fischer2Lars Pape3Thorsten Wiech4Markus J. Kemper5Burkhard Tönshoff6Jun Oh7Raphael Schild8Department of Pediatric Nephrology, Pediatric Hepatology and Pediatric Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyDepartment of Pediatric Nephrology, Pediatric Hepatology and Pediatric Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyDepartment of Hepatobiliary Surgery and Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyDepartment of Pediatrics II, University Hospital of Essen, University of Essen-Duisburg, Essen, GermanyInstitute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyDepartment of Pediatrics, Asklepios Klinik Nord-Heidberg, Hamburg, GermanyDepartment of Pediatrics I, University Children’s Hospital Heidelberg, Heidelberg, GermanyDepartment of Pediatric Nephrology, Pediatric Hepatology and Pediatric Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyDepartment of Pediatric Nephrology, Pediatric Hepatology and Pediatric Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyBackgroundEarly onset de novo focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized treatment protocols have not yet been established.Case descriptionWe describe a boy with early onset de novo FSGS in the transplanted kidney and non-selective glomerular proteinuria (maximum albumin-to-creatinine ratio of 3.8 g/g; normal range, ≤0.03 g/g creatinine). Manifestation occurred at 30 days posttransplant and was accompanied by a significant graft dysfunction (eGFR 61 ml/min per 1.73 m2). Treatment with 25 sessions of plasmapheresis over 14 weeks and three consecutive days of methylprednisolone pulse therapy (10 mg/kg per day) followed by oral prednisolone as rejection prophylaxis (3.73 mg/m2 per day) led to sustained remission of proteinuria (albumin-to-creatinine ratio of 0.028 g/g) and normalization of graft function (eGFR 92 ml/min per 1.73 m2) after 14 weeks. The follow-up period was 36 months.ConclusionsThis case underlines the efficacy of immunosuppressive and antibody eliminating therapy in early onset de novo FSGS after kidney transplantation.https://www.frontiersin.org/articles/10.3389/fped.2023.1280521/fullde novo focal segmental glomerulosclerosiskidney transplantpediatric donorproteinuriaplasmapharesis
spellingShingle Karla Carvajal Abreu
Sebastian Loos
Lutz Fischer
Lars Pape
Thorsten Wiech
Markus J. Kemper
Burkhard Tönshoff
Jun Oh
Raphael Schild
Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
Frontiers in Pediatrics
de novo focal segmental glomerulosclerosis
kidney transplant
pediatric donor
proteinuria
plasmapharesis
title Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
title_full Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
title_fullStr Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
title_full_unstemmed Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
title_short Case report: Early onset de novo FSGS in a child after kidney transplantation—a successful treatment
title_sort case report early onset de novo fsgs in a child after kidney transplantation a successful treatment
topic de novo focal segmental glomerulosclerosis
kidney transplant
pediatric donor
proteinuria
plasmapharesis
url https://www.frontiersin.org/articles/10.3389/fped.2023.1280521/full
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