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Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation

Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 month...

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Autores principales: Bouatou, Yassine, Bacchi, Véronique Frémeaux, Villard, Jean, Moll, Solange, Martin, Pierre-Yves, Hadaya, Karine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4946455/
https://www.ncbi.nlm.nih.gov/pubmed/27500215
http://dx.doi.org/10.1097/TXD.0000000000000518
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author Bouatou, Yassine
Bacchi, Véronique Frémeaux
Villard, Jean
Moll, Solange
Martin, Pierre-Yves
Hadaya, Karine
author_facet Bouatou, Yassine
Bacchi, Véronique Frémeaux
Villard, Jean
Moll, Solange
Martin, Pierre-Yves
Hadaya, Karine
author_sort Bouatou, Yassine
collection PubMed
description Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no “humps” on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the “at-risk” complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis.
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spelling pubmed-49464552016-08-05 Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation Bouatou, Yassine Bacchi, Véronique Frémeaux Villard, Jean Moll, Solange Martin, Pierre-Yves Hadaya, Karine Transplant Direct Original Clinical Science Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no “humps” on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the “at-risk” complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis. Lippincott Williams & Wilkins 2015-03-26 /pmc/articles/PMC4946455/ /pubmed/27500215 http://dx.doi.org/10.1097/TXD.0000000000000518 Text en Copyright © 2015 The Authors. Transplantation Direct. Published by Wolters Kluwer Health, Inc. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License, where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/3.0.
spellingShingle Original Clinical Science
Bouatou, Yassine
Bacchi, Véronique Frémeaux
Villard, Jean
Moll, Solange
Martin, Pierre-Yves
Hadaya, Karine
Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title_full Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title_fullStr Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title_full_unstemmed Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title_short Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation: C3-Glomerulonephritis as an Initial Presentation
title_sort atypical hemolytic uremic syndrome recurrence after renal transplantation: c3-glomerulonephritis as an initial presentation
topic Original Clinical Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4946455/
https://www.ncbi.nlm.nih.gov/pubmed/27500215
http://dx.doi.org/10.1097/TXD.0000000000000518
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