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Pediatric Nephrology

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01-02-2019 | Brief Report

Long-term outcomes after early treatment with rituximab for Japanese children with cyclosporine- and steroid-resistant nephrotic syndrome

Authors: Shuichiro Fujinaga, Tomohiko Nishino, Chisato Umeda, Yuji Tomii, Yoshitaka Watanabe, Koji Sakuraya

Published in: Pediatric Nephrology | Issue 2/2019

Abstract

Background

Although rituximab (RTX) may be effective treatment in children with nephrotic syndrome who are resistant to cyclosporine A and steroid (CsA-SRNS), long-term outcomes after B cell depleting therapy remain unclear.

Case-diagnosis/treatment

We retrospectively reviewed the clinical courses (median follow-up, 5.1 years) of six CsA-SRNS children (three boys; median age at RTX, 4.2 years) unresponsive to oral cyclosporine combined with ≥ 2 courses of intravenous methylprednisolone pulses, who received RTX within 6 months after disease onset (median 11 weeks). After initial RTX treatment (median two doses of 375 mg/m²) followed by retreatment with intravenous methylprednisolone pulses and/or high-dose prednisolone, all patients achieved complete remission at a median of 158 days. Although 17 relapses occurred in five patients during follow-up, all but one patient became steroid sensitive. Severe neutropenia and hypogammaglobulinemia developed in two and four patients, respectively. However, no life-threatening infections were identified in the cohort. At last visit (median age, 11.3 years), all patients maintained complete remission without renal insufficiency.

Conclusions

Although late-onset adverse events should be considered, particularly for young patients, early RTX treatment may have positive outcomes in children with CsA-SRNS in the long term.

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Title: Long-term outcomes after early treatment with rituximab for Japanese children with cyclosporine- and steroid-resistant nephrotic syndrome
Authors: Shuichiro Fujinaga
Tomohiko Nishino
Chisato Umeda
Yuji Tomii
Yoshitaka Watanabe
Koji Sakuraya
Publication date: 01-02-2019
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 2/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-018-4145-6

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Long-term outcomes after early treatment with rituximab for Japanese children with cyclosporine- and steroid-resistant nephrotic syndrome

Abstract

Background

Case-diagnosis/treatment

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Case-diagnosis/treatment

Conclusions

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