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Benign and malignant proliferation in idiopathic nephrotic syndrome: a French cohort study

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Abstract

Background

There seems to be a possible link between nephrotic syndrome (NS) and lymphoproliferative syndrome, but it remains poorly understood.

Methods

This multicentric and retrospective study focuses on children, who developed idiopathic NS and malignant or benign proliferation between 2000 and 2021.

Results

Eleven patients were included, with a median age of 4 years. Only one had a steroid-resistant nephrotic syndrome (SRNS). The maintenance therapy before the proliferation was in majority tacrolimus or mycophenolate mofetil (MMF), but three patients did not receive treatments. The proliferation was mainly a Hodgkin’s lymphoma (45%) or a lymphoproliferative disease (36%), in a median time after the NS of two years. Viruses were found in seven cases (EBV in five cases and HHV-8 in two).

Conclusion

The association between proliferative syndrome and idiopathic NS may not be fortuitous, possibly with a common lymphocytic disturbance. Genetic analyses could improve the comprehension of these manifestations in the future.

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Abbreviations

AIDS:

Acquired immunodeficiency syndrome

DNA:

Deoxyribonucleic acid

EBV:

Epstein-Barr virus

EBV-SMT:

Epstein-Barr virus-associated smooth muscle tumors

HLA:

Human leukocyte antigen

HHV-8:

Human herpesvirus-8

MCD:

Minimal change disease

MMF:

Mycophenolate mofetil

NFAT-5:

Nuclear factor of activated T cell 5

NS:

Nephrotic syndrome

OIIA-LPDs:

Other iatrogenic immunodeficiency-associated lymphoproliferative disorder

PTLD:

Post-transplant lymphoproliferative disorder

TonEBP:

Tonicity-responsive enhancer binding protein

VUS:

Variant of uncertain significance

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Author notes

  1. Thomas Simon and Jérôme Harambat contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatric Internal Medicine, Rheumatology and Nephrology, Centre De Référence Des Maladies Rénales Rares du Sud-Ouest, SoRare, Toulouse University Hospital, Toulouse, France

    Clara Cébron, Stéphane Decramer & Thomas Simon

  2. Pediatric Department, Grenoble University Hospital, Grenoble, France

    Clara Cébron

  3. Department of Pediatrics, Pediatric Nephrology Unit, Centre De Référence Des Maladies Rénales Rares du Sud-Ouest, SoRare, Bordeaux University Hospital, Bordeaux, France

    Astrid Godron-Dubrasquet & Jérôme Harambat

  4. Department of Pediatrics, Pediatric Oncology Hematology Unit, Bordeaux University Hospital, Bordeaux, France

    Nathalie Aladjidi

  5. Pediatric Department, Nantes University Hospital, Nantes, France

    Gwenaelle Roussey & Emma Allain-Launay

  6. Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, Imagine Institute, Paris University, Necker Hospital, APHP, Paris, France

    Olivia Boyer & Marina Avramescu

  7. Department of Pediatric Nephrology, Robert Debré Hospital, APHP, Paris, France

    Veronique Baudouin

  8. Department of Pediatric Nephrology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

    Joelle Terzic

  9. Imagine Institute, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM, UMR 1163, Université de Paris, Paris, France

    Frédéric Rieux-Laucat

  10. Centre De Référence Des Maladies Rénales Rares du Sud-Ouest, SoRare, Membre du réseau européen ERKNet, Toulouse, France

    Stéphane Decramer & Thomas Simon

  11. INSERM U1048, Institute of Cardiovascular and Metabolic Diseases, Toulouse, France

    Stéphane Decramer

Authors
  1. Clara Cébron
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  2. Astrid Godron-Dubrasquet
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  3. Nathalie Aladjidi
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  4. Gwenaelle Roussey
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  5. Olivia Boyer
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  7. Veronique Baudouin
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  8. Joelle Terzic
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  9. Emma Allain-Launay
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  10. Frédéric Rieux-Laucat
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  11. Stéphane Decramer
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  12. Thomas Simon
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  13. Jérôme Harambat
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Corresponding author

Correspondence to Thomas Simon.

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Cébron, C., Godron-Dubrasquet, A., Aladjidi, N. et al. Benign and malignant proliferation in idiopathic nephrotic syndrome: a French cohort study. Pediatr Nephrol 37, 1837–1843 (2022). https://doi.org/10.1007/s00467-021-05386-0

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  • DOI: https://doi.org/10.1007/s00467-021-05386-0

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