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Pediatr Nephrol. 2019 Jan;34(1):177-181. doi: 10.1007/s00467-018-4095-z. Epub 2018 Sep 28.

B cell phenotype in pediatric idiopathic nephrotic syndrome.

Author information

1
Laboratory of Nephrology, Department of Rare Diseases, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy. manuela.colucci@opbg.net.
2
Department of Laboratories, Immunology Research Area - Unit of Diagnostic Immunology, Unit of B-cell Pathophysiology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
3
Laboratory of Nephrology, Department of Rare Diseases, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
4
Division of Nephrology, Department of Pediatric Subspecialties, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.

Abstract

BACKGROUND:

A pathogenic role of B cells in non-genetic nephrotic syndrome has been suggested by the efficacy of rituximab, a B cell depleting antibody, in maintaining a prolonged remission. However, little information is available on B cell homeostasis in nephrotic syndrome patients.

METHODS:

We retrospectively analyzed by flow cytometry the distribution of different B cell subpopulations in 107 steroid-sensitive and in 6 genetic steroid-resistant nephrotic syndrome pediatric patients, compared with age- and sex-matched controls.

RESULTS:

Fifty-one steroid-sensitive patients at disease onset, before starting immunosuppression, presented significantly increased levels of total, transitional, memory, and switched memory B cells compared to controls. Oral immunosuppression strongly affected transitional and mature B cell levels in 27 patients in relapse and also in 29 patients in remission, whereas memory B cells were significantly higher compared to controls during relapse, despite the immunosuppressive treatment, and were normalized only in patients in remission. Children with genetic forms of steroid-resistant nephrotic syndrome presented no differences in B cell profile from controls.

CONCLUSIONS:

Our study indicates that memory B cells, more than other B cell subsets, are increased and appear to be pathogenically relevant in steroid-sensitive nephrotic syndrome pediatric patients.

KEYWORDS:

B cells; Children; Immunosuppressive treatment; Nephrotic syndrome

PMID:
30267238
DOI:
10.1007/s00467-018-4095-z

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