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Clinical and Experimental Nephrology

, Volume 19, Issue 1, pp 6–33 | Cite as

Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy

  • Kenji IshikuraEmail author
  • Shinsuke Matsumoto
  • Mayumi Sako
  • Kazushi Tsuruga
  • Koichi Nakanishi
  • Koichi Kamei
  • Hiroshi Saito
  • Shuichiro Fujinaga
  • Yuko Hamasaki
  • Hiroko Chikamoto
  • Yasufumi Ohtsuka
  • Yasuhiro Komatsu
  • Toshiyuki Ohta
  • Takuhito Nagai
  • Hiroshi Kaito
  • Shuji Kondo
  • Yohei Ikezumi
  • Seiji Tanaka
  • Yoshitsugu Kaku
  • Kazumoto Iijima
Guideline

Introduction

Nephrotic syndrome is a disorder characterized by severe proteinuria, hypoproteinemia, and generalized edema resulting from damage to the glomerular basement membrane. In Western countries, nephrotic syndrome affects 2 of 100,000 children per year [1]. In Japan, approximately 1,300 new cases per year of pediatric nephrotic syndrome are reported to the Medical Aid for Specific Chronic Disease of Children and the disease develops in 5 of 100,000 children per year. Approximately 90 % of the cases of pediatric nephrotic syndrome are idiopathic, or of unknown cause. The first-line treatment for an initial episode of pediatric idiopathic nephrotic syndrome is oral steroid therapy, which leads to remission in approximately 80 % of cases (steroid-sensitive nephrotic syndrome) [2]. However, 80 % of children with steroid-sensitive nephrotic syndrome experience one or more relapses, [3] and 50 % of these children have frequent relapses [4]. Those with frequently relapsing nephrotic...

Keywords

Tacrolimus Nephrotic Syndrome Mycophenolate Mofetil Posterior Reversible Encephalopathy Syndrome Idiopathic Nephrotic Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

The development of this guideline was solely supported by the society budget of the Japanese Society for Pediatric Nephrology. All members engaged in the guideline development submitted Conflicts of Interest declaration forms, which are retained by the Japanese Society for Pediatric Nephrology. To avoid the influence of any conflicts of interest on the contents of this guideline, discussions were made in an open manner using a mailing list system. Opinions of multiple reviewers (councilor board members of the Japanese Society for Pediatric Nephrology and an epidemiologist as an outside reviewer) and public comments were used to refine the guideline.

Kazumoto Iijima, Kenji Ishikura, and Yoshitsugu Kaku played an essential role in scientific discussion and preparation and review of the manuscript. The rest of the authors all contributed equally to the work. The authors thank for the contribution of Ms. Fujimi Kawai and Ms. Satomi Kojima, The Japan Medical Library Association, for expert consultation on the development of the guideline; Mr. Kyoshiro Yanagisawa and Ms. Yumi Yanagisawa for advice from a viewpoint of a patient and its guardian; Dr. Kaori Kikunaga, Tokyo Metropolitan Children’s Medical Center, for clerical and coordination duties; ASCA Corporation for translation and editorial supports in the preparation of the manuscript; and Dr. Noriko Kojimahara, Tokyo Women’s Medical University, Dr. Shuichi Ito, the National Center for Child Health and Development, and Dr. Shoji Kagami, the University of Tokushima, for proper and thorough review of the guideline.

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Copyright information

© Japanese Society of Nephrology 2015

Authors and Affiliations

  • Kenji Ishikura
    • 1
    Email author
  • Shinsuke Matsumoto
    • 2
  • Mayumi Sako
    • 3
  • Kazushi Tsuruga
    • 4
  • Koichi Nakanishi
    • 5
  • Koichi Kamei
    • 6
  • Hiroshi Saito
    • 7
  • Shuichiro Fujinaga
    • 8
  • Yuko Hamasaki
    • 9
  • Hiroko Chikamoto
    • 10
  • Yasufumi Ohtsuka
    • 11
  • Yasuhiro Komatsu
    • 12
  • Toshiyuki Ohta
    • 13
  • Takuhito Nagai
    • 14
  • Hiroshi Kaito
    • 15
  • Shuji Kondo
    • 16
  • Yohei Ikezumi
    • 17
  • Seiji Tanaka
    • 18
  • Yoshitsugu Kaku
    • 19
  • Kazumoto Iijima
    • 20
  1. 1.Department of NephrologyTokyo Metropolitan Children’s Medical CenterFuchuJapan
  2. 2.Department of PediatricsMatsudo City Hospital Children’s Medical CenterChibaJapan
  3. 3.Division for Clinical Trials, Department of Development Strategy, Center for Social and Clinical ResearchNational Research Institute for Child Health and DevelopmentTokyoJapan
  4. 4.Department of PediatricsHirosaki University Graduate School of MedicineHirosakiJapan
  5. 5.Department of PediatricsWakayama Medical UniversityWakayamaJapan
  6. 6.Department of Nephrology and RheumatologyNational Center for Child Health and DevelopmentTokyoJapan
  7. 7.Department of PediatricsNihon University School of MedicineTokyoJapan
  8. 8.Division of NephrologySaitama Children’s Medical CenterSaitamaJapan
  9. 9.Department of Pediatric NephrologyToho University Faculty of MedicineTokyoJapan
  10. 10.Department of Pediatric Nephrology, School of MedicineTokyo Women’s Medical UniversityTokyoJapan
  11. 11.Department of Pediatrics, Faculty of MedicineSaga UniversitySagaJapan
  12. 12.Division of Nephrology, Department of MedicineSt. Luke’s International HospitalTokyoJapan
  13. 13.Department of Pediatric NephrologyHiroshima Prefectural HospitalHiroshimaJapan
  14. 14.Department of NephrologyAichi Children’s Health and Medical CenterAichiJapan
  15. 15.Department of PediatricsKobe University Graduate School of MedicineKobeJapan
  16. 16.Department of Pediatrics, Institute of Health BioscienceTokushima UniversityTokushimaJapan
  17. 17.Department of PediatricsNiigata University Medical and Dental HospitalNiigataJapan
  18. 18.Department of Pediatrics and Child HealthKurume University Medical CenterFukuokaJapan
  19. 19.Department of NephrologyFukuoka Children’s HospitalFukuokaJapan
  20. 20.Department of PediatricsKobe University Graduate School of MedicineKobeJapan

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