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Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis

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Abstract

Background

Cyclosporine has improved remission rates in children with steroid-resistant nephrotic syndrome (SRNS). However, little prospective long-term follow-up data is available.

Methods

We prospectively followed and analyzed 5-year outcomes of all 35 patients enrolled in our previous prospective multicenter trial with cyclosporine and steroids in children with SRNS. At enrollment, 23 cases were classified as minimal change (MC), five as diffuse mesangial proliferation (DMP), and seven as focal segmental glomerulosclerosis (FSGS).

Results

Renal survival at 5 years (median 7.7 years) was 94.3 %. Patient status was complete remission (CR) in 31 (88.6 %) (MC/DMP, 25; FSGS, 6); partial remission in one (FSGS); and non-remission in three (MC/DMP), including chronic kidney disease and end-stage kidney disease in one each. Among 31 patients with CR, 22 (71.0 %) were receiving treatment with immunosuppressants at 5 years, including cyclosporine in 19, and seven of these 22 continued to show frequent relapse. Response to cyclosporine at 4 months predicted 5-year outcome in 31 of 35 patients.

Conclusions

Although SRNS treatment with cyclosporine provides high renal survival and remission rates, many children require ongoing immunosuppression. Management has advanced from the prevention of end-stage kidney disease to the long-term maintenance of remission and management of relapse after induction therapy.

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Acknowledgments

This study was supported in part by the Kidney Foundation, Japan. The results presented in this paper have not been published previously in whole or part, except in abstract form at the 43rd Annual American Society of Nephrology Conference.

The authors would like to thank Drs. Goto M (Tokyo), Hamada R (Tokyo), Harada T (Kanagawa), Hasegawa K (Tokyo), Hataya H (Tokyo), Hayakawa H (Niigata), Ikeda M (Tokyo), Kaito H (Hyogo), Kamei K (Tokyo), Kitayama H (Shizuoka), Kobayashi A (Hyogo), Kodama S (Fukuoka), Miura K (Tokyo), Nakamura T (Kanagawa), Nozu K (Hyogo), Ochiai R (Tokyo), Sakai T (Tokyo), Sekine T (Tokyo), Suzuki R (Tokyo), Tanaka R (Hyogo), Wada N (Shizuoka), Wakaki H (Tokyo), and Yoshidome K (Kagoshima) of the Japanese Study Group of Renal Disease in Children for their contributions to the study.

Conflict of interest statement

Norishige Yoshikawa has received research grants from Novartis, Japan.

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Authors and Affiliations

  1. Department of Nephrology, Tokyo Metropolitan Children’s Medical Center, Fuchu, Japan

    Yuko Hamasaki, Kenji Ishikura & Masataka Honda

  2. Department of Pediatric Nephrology, Toho University Omori Medical Center, 6-11-1, Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan

    Yuko Hamasaki

  3. Department of Pediatrics, Wakayama Medical University, Wakayama, Japan

    Norishige Yoshikawa & Koichi Nakanishi

  4. Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

    Hitoshi Nakazato

  5. Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan

    Satoshi Sasaki

  6. Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan

    Kazumoto Iijima

  7. Department of Pediatrics, Fussa Hospital, Fussa, Japan

    Takeshi Matsuyama

  8. Division of Nephrology and Rheumatology, National Center for Child Health and Development, Setagaya-ku, Japan

    Shuichi Ito

  9. Department of Clinical Research, Tokyo Metropolitan Children’s Medical Center, Fuchu, Japan

    Tetsuji Kaneko

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  1. Yuko Hamasaki
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  2. Norishige Yoshikawa
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  11. Masataka Honda
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Correspondence to Yuko Hamasaki.

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Hamasaki, Y., Yoshikawa, N., Nakazato, H. et al. Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis. Pediatr Nephrol 28, 765–771 (2013). https://doi.org/10.1007/s00467-012-2393-4

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  • DOI: https://doi.org/10.1007/s00467-012-2393-4

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