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Multicenter Study
. 2022 Aug;37(8):1837-1843.
doi: 10.1007/s00467-021-05386-0. Epub 2022 Jan 10.

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

Clara Cébron  1   2 , Astrid Godron-Dubrasquet  3 , Nathalie Aladjidi  4 , Gwenaelle Roussey  5 , Olivia Boyer  6 , Marina Avramescu  6 , Veronique Baudouin  7 , Joelle Terzic  8 , Emma Allain-Launay  5 , Frédéric Rieux-Laucat  9 , Stéphane Decramer  1   10   11 , Thomas Simon #  12   13 , Jérôme Harambat #  3
Affiliations
Multicenter Study

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

Clara Cébron et al. Pediatr Nephrol. 2022 Aug.

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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Keywords: Children; Lymphoproliferation; Nephrotic syndrome; Smooth muscle tumors.

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Supplementary concepts