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Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial

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Abstract

Background

To investigate the long-term outcome in children with frequently relapsing nephrotic syndrome (FRNS) we conducted a follow-up of a previous randomized controlled trial (RCT) 10 years after the initiation of the treatment protocol.

Methods

We previously conducted an RCT on the efficacy of cyclosporine for treating children with FRNS. After 2 years of treatment, a recommended a management protocol of steroids, and immunosuppressants was provided.

Results

Valid information was available for 46 of the 56 patients (82.1 %) enrolled in the original RCT. The median follow-up period was 10.3 years from the start of protocol treatment with cyclosporine. At last follow-up (mean age 18.7 years), only ten patients (21.7 %) showed disease-free remission (no relapse for at least 2 years). In contrast, 23 (50.0 %) continued to relapse frequently or were on immunosuppressants, eight patients (17.4 %) had infrequent relapses without immunosuppressants. Adverse effects attributable to treatment included short stature (6 patients), osteoporosis (six patients), obesity (4 patients), cataracts (3 patients) and hypertension (3 patients). No lethal event or renal dysfunction occurred during follow-up.

Conclusions

This 10-year follow-up study shows that most children with FRNS experience relapses after 2 years of cyclosporine treatment, in adolescence and into adulthood. Outcomes in terms of life expectancy and renal function are favorable.

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Acknowledgments

The authors would like to thank Drs. Yoshinori Araki (Hokkaido), Midori Awazu (Tokyo), Akio Furuse (Kumamoto), Miwa Goto (Yamanashi), Riku Hamada (Tokyo), Junya Hashimoto (Tokyo), Ken Hatae (Fukuoka), Hiroshi Hataya (Tokyo), Misako Hiramatsu (Oita), Ryugo Hiramoto (Chiba), Isho Izumi (Ibaraki), Yoshitsugu Kaku (Fukuoka), Aiju Kameda (Hyogo), Kentaro Kamezaki (Fukuoka), Koichi Kamei (Tokyo), Hidekazu Kamitsuji (Nara), Kosaku Kitagawa (Osaka), Yukiko Matayoshi (Miyazaki), Shinsuke Matsumoto (Chiba), Toshinori Minato (Hyogo), Hajime Miyamoto (Hyogo), Masamitsu Nishino (Osaka), Aya Nomura (Tokyo), Kandai Nozu (Hyogo), Yoko Ohwada (Tochigi), Shojiro Okamoto (Tokyo), Tomoyuki Sakai (Shiga), Mayumi Sako (Tokyo), Tadashi Sato (Saga), Kazuki Tamura (Ibaraki), Ryojiro Tanaka (Hyogo), Yuriko Tanaka (Saitama), Yasushi Tsutsumi (Fukuoka), Kaori Yoneda (Kumamoto), Megumi Yoshimura-Furuhata (Nagano) of the Japanese Study Group of Renal Disease in Children for their contributions to the study. The authors would also like to thank to Ms. Sachiko Kawabe for her support.

Financial disclosure

This study was supported by the Kidney Foundation, Japan.

Kenji Ishikura has received lecture fees from Novartis Pharma K.K. and Asahi Kasei Pharma Corporation. Norishige Yoshikawa has received grants from Novartis Pharma K.K. and Asahi Kasei Pharma Corporation and has also received lecture fees from Novartis Pharma K.K. and Asahi Kasei Pharma Corporation. Koichi Nakanishi has received lecture fees from Novartis Pharma K.K., Asahi Kasei Pharma Corporation, and Astellas Pharma. Takeshi Matsuyama has received lecture fees from Asahi Kasei Pharma Corporation and Terumo Medical Corporation. Shuichi Ito received lecture fees from Asahi Kasei Pharma Corporation, Novartis Pharma K.K., and Chugai Pharmaceutical Co. Ltd. Yuko Hamasaki has received research grants from Novartis Pharma K.K., and lecture fees from Novartis Pharma K.K., Astellas Pharma, and Pfizer Japan. Kazumoto Iijima has received grants from Takeda Pharmaceutical Co., Ltd., Asahi Kasei Pharma Corporation, and Novartis Pharma K.K., and lecture fees from Novartis Pharma K.K. and Asahi Kasei Pharma Corporation. Masataka Honda has received lecture fees from Novartis Pharma and Asahi Kasei Pharma Corporation. The other authors have no conflicts of interest to declare.

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Correspondence to Kenji Ishikura.

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Ishikura, K., Yoshikawa, N., Nakazato, H. et al. Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial. Pediatr Nephrol 30, 459–468 (2015). https://doi.org/10.1007/s00467-014-2955-8

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  • DOI: https://doi.org/10.1007/s00467-014-2955-8

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