Skip to main content
SpringerLink
Log in

High incidence of idiopathic nephrotic syndrome in East Asian children: a nationwide survey in Japan (JP-SHINE study)

  • Original article
  • Published:
Clinical and Experimental Nephrology Aims and scope Submit manuscript

Abstract

Background

Little is known regarding the epidemiology of idiopathic nephrotic syndrome (INS) in East Asia. Previous studies have suggested higher incidence of INS in Asian children, though decreasing trend of its incidence has also been shown.

Methods

We conducted a nationwide study of Japanese children aged 6 months to 15 years with INS. Children who were newly diagnosed with INS between 1 January 2010 and 31 December 2012 were eligible. Children with congenital nephrotic syndrome or nephrotic syndrome secondary to nephritis were excluded.

Results

A total of 2099 children were initially diagnosed with INS and were followed for up to 4 years. The estimated incidence of INS was 6.49 cases/100,000 children per year, without clear correlation with geographical region. The male:female ratio was 1.9 and approximately 50 % of children were <5 years old at diagnosis. During the 1–4 years follow-up, 32.7 % developed frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.

Conclusions

Based on our nationwide survey, the incidence of INS in Japanese children is approximately 3–4 times higher than that in Caucasians. However, the male:female ratio and the age at onset were similar to those in previous studies. We are now planning a prospective cohort study to examine the course of INS in Japan.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003;362:629–39.

    Article  PubMed  Google Scholar 

  2. van Husen M, Kemper MJ. New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol. 2011;26:881–92.

    Article  PubMed  Google Scholar 

  3. Kengne-Wafo S, Massella L, Diomedi-Camassei F, Gianviti A, Vivarelli M, Greco M, Stringini GR, Emma F. Risk factors for cyclosporin A nephrotoxicity in children with steroid-dependant nephrotic syndrome. Clin J Am Soc Nephrol. 2009;4:1409–16.

    Article  CAS  PubMed  Google Scholar 

  4. Pravitsitthikul N, Willis NS, Hodson EM, Craig JC. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database Syst Rev. 2013;10:CD002290.

    Google Scholar 

  5. Warady BA, Chadha V. Chronic kidney disease in children: the global perspective. Pediatr Nephrol. 2007;22:1999–2009.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Harambat J, van Stralen KJ, Kim JJ, Tizard EJ. Epidemiology of chronic kidney disease in children. Pediatr Nephrol. 2012;27:363–73.

    Article  PubMed  Google Scholar 

  7. Wong W. Idiopathic nephrotic syndrome in New Zealand children, demographic, clinical features, initial management and outcome after twelve-month follow-up: results of a three-year national surveillance study. J Paediatr Child Health. 2007;43:337–41.

    Article  PubMed  Google Scholar 

  8. El Bakkali L, Rodrigues Pereira R, Kuik DJ, Ket JC, van Wijk JA. Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature. Pediatr Nephrol. 2011;26:1241–6.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Chang JW, Tsai HL, Yang LY, Chen TJ. Epidemiology and predictors of end-stage renal disease in Taiwanese children with idiopathic nephrotic syndrome. J Epidemiol. 2012;22:517–22.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Ernould S, Godron A, Nelson JR, Rigothier C, Llanas B, Harambat J. Idiopathic nephrotic syndrome in children: incidence, clinical presentation, and outcome in the county of Gironde, France. Arch Pediatr. 2011;18:522–8 (in French).

    Article  CAS  PubMed  Google Scholar 

  11. Feehally J, Kendell NP, Swift PG, Walls J. High incidence of minimal change nephrotic syndrome in Asians. Arch Dis Child. 1985;60:1018–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol. 2001;16:1040–4.

    Article  CAS  PubMed  Google Scholar 

  13. Sharples PM, Poulton J, White RH. Steroid responsive nephrotic syndrome is more common in Asians. Arch Dis Child. 1985;60:1014–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Ishikura K, Matsumoto S, Sako M, Tsuruga K, Nakanishi K, Kamei K, Saito H, Fujinaga S, Hamasaki Y, Chikamoto H, Ohtsuka Y, Komatsu Y, Ohta T, Nagai T, Kaito H, Kondo S, Ikezumi Y, Tanaka S, Kaku Y, Iijima K. Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy. Clin Exp Nephrol. 2015;19:6–33.

    Article  PubMed  Google Scholar 

  15. Kaku Y, Ohtsuka Y, Komatsu Y, Ohta T, Nagai T, Kaito H, Kondo S, Ikezumi Y, Tanaka S, Matsumoto S, Sako M, Tsuruga K, Nakanishi K, Kamei K, Saito H, Fujinaga S, Hamasaki Y, Chikamoto H, Ishikura K, Iijima K. Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: general therapy. Clin Exp Nephrol. 2015;19:34–53.

    Article  PubMed  Google Scholar 

  16. Hashimoto S, Fukutomi K, Nagai M, Nakamura Y, Yanagawa H, Sasaki R, Ohno Y. A note on methods for estimating the number of patients in the nationwide epidemiological survey on intractable diseases. Nihon Koshu Eisei Zasshi. 1990;37:768–74 (in Japanese).

    CAS  PubMed  Google Scholar 

  17. Srivastava T, Simon SD, Alon US. High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol. 1999;13:13–8.

    Article  CAS  PubMed  Google Scholar 

  18. Zaki M, Helin I, Manandhar DS, Hunt MC, Khalil AF. Primary nephrotic syndrome in Arab children in Kuwait. Pediatr Nephrol. 1989;3:218–20 (discussion 221).

    Article  CAS  PubMed  Google Scholar 

  19. Jin DK, Kohsaka T, Tanaka M, Abe J, Kobayashi N. Human leukocyte antigens in childhood idiopathic nephrotic syndrome. Acta Paediatr Jpn. 1991;33:709–13.

    Article  CAS  PubMed  Google Scholar 

  20. Yoshikawa N, Nakanishi K, Sako M, Oba MS, Mori R, Ota E, Ishikura K, Hataya H, Honda M, Ito S, Shima Y, Kaito H, Nozu K, Nakamura H, Igarashi T, Ohashi Y, Iijima K. A multicenter randomized trial indicates initial prednisolone treatment for childhood nephrotic syndrome for 2 months is not inferior to six-month treatment. Kidney Int. 2015;87:225–32.

    Article  CAS  PubMed  Google Scholar 

  21. Nakanishi K, Iijima K, Ishikura K, Hataya H, Nakazato H, Sasaki S, Honda M, Yoshikawa N. Two-year outcome of the ISKDC regimen and frequent-relapsing risk in children with idiopathic nephrotic syndrome. Clin J Am Soc Nephrol. 2013;8:756–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Sinha A, Saha A, Kumar M, Sharma S, Afzal K, Mehta A, Kalaivani M, Hari P, Bagga A. Extending initial prednisolone treatment in a randomized control trial from 3 to 6 months did not significantly influence the course of illness in children with steroid-sensitive nephrotic syndrome. Kidney Int. 2015;87:217–24.

    Article  CAS  PubMed  Google Scholar 

  23. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol. 1997;8:769–76.

    CAS  PubMed  Google Scholar 

  24. Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P, Lesavre P, Chauveau D, Knebelmann B, Broyer M, Grunfeld JP, Niaudet P. Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis. 2003;41:550–7.

    Article  PubMed  Google Scholar 

  25. Ruth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ. Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202–7.

    Article  PubMed  Google Scholar 

  26. Ishikura K, Yoshikawa N, Nakazato H, Sasaki S, Nakanishi K, Matsuyama T, Ito S, Hamasaki Y, Yata N, Ando T, Iijima K, Honda M. Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial. Pediatr Nephrol. 2015;30:459–68.

    Article  PubMed  Google Scholar 

  27. Hamasaki Y, Yoshikawa N, Nakazato H, Sasaki S, Iijima K, Nakanishi K, Matsuyama T, Ishikura K, Ito S, Kaneko T, Honda M. Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis. Pediatr Nephrol. 2013;28:765–71.

    Article  PubMed  Google Scholar 

  28. Dossier C, Sellier-Leclerc AL, Rousseau A, Michel Y, Gautheret-Dejean A, Englender M, Madhi F, Charbit M, Ulinski T, Simon T, Jacqz-Aigrain E, Deschenes G. Prevalence of herpesviruses at onset of idiopathic nephrotic syndrome. Pediatr Nephrol. 2014;29:2325–31.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

Information on all hospitals, institutions, and pediatricians was extracted from the “Medical database” supported by Nihon Ultmarc Inc. We thank Nicholas Smith, PhD, of Edanz Group Ltd., for providing language editorial support in the preparation of the manuscript. We also thank everyone who contributed data to this study, including all of the anonymous participants.

Author information

Authors and Affiliations

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

    Kaori Kikunaga, Kenji Ishikura, Chikako Terano & Masataka Honda

  2. Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan

    Kenji Ishikura, Mai Sato & Shuichi Ito

  3. Department of Health and Welfare Bureau, Health and Welfare Bureau Health Center of Kawasaki City, Kanagawa, Japan

    Fumiyo Komaki

  4. Department of Pediatric Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan

    Yuko Hamasaki

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

    Satoshi Sasaki

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

    Kazumoto Iijima

  7. Clinical Research Center, Wakayama Medical University, Wakayama, Japan

    Norishige Yoshikawa & Koichi Nakanishi

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

    Hitoshi Nakazato

  9. Department of Pediatrics, Fussa Hospital, Tokyo, Japan

    Takeshi Matsuyama

  10. Japan Clinical Research Support Unit, Tokyo, Japan

    Takashi Ando

  11. Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Yokohama, Japan

    Shuichi Ito

Authors
  1. Kaori Kikunaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kenji Ishikura
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Chikako Terano
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Mai Sato
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Fumiyo Komaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yuko Hamasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Satoshi Sasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Kazumoto Iijima
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Norishige Yoshikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Koichi Nakanishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Hitoshi Nakazato
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Takeshi Matsuyama
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Takashi Ando
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Shuichi Ito
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Masataka Honda
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

On behalf of the Japanese Pediatric Survey Holding Information of NEphrotic syndrome (JP-SHINE) study of the Japanese Study Group of Renal Disease in Children

Corresponding author

Correspondence to Kenji Ishikura.

Ethics declarations

Financial disclosure

This study was supported by Asahi Kasei Pharma Corporation, Astellas Pharma, Fuso Pharmaceutical Industries, Ltd, JMS Co., Ltd., Novartis Pharma K.K., and Zenyaku Kogyo Co., Ltd.

Conflict of interest

Norishige Yoshikawa has received research funding from Novartis Pharma K.K. and Asahi Kasei Pharma Corporation. Kazumoto Iijima has received research funding from Daiichi Sankyo, Co., Ltd., Clio, Co., Ltd., Sysmex Corporation, and manuscript fees from Chugai Pharmaceutical Co., Ltd. Yuko Hamasaki belongs to an endowed department sponsored by Asahi Kasei Pharma Corporation, Novartis Pharma K.K., Chugai Pharmaceutical Co., and Astellas Pharma.

Ethics

The present study was conducted in accordance with the ethical principles set out in the Declarations of Helsinki, and with the ethical guidelines for epidemiological studies issued by the Ministry of Health, Labour and Welfare in Japan. The study was approved by a central ethics board (National Center for Child Health and Development; approval number 702) before commencing the study. Because all data were reported in a retrospective manner using patient charts, informed consent was not obtained in accordance with the above guidelines.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (TIFF 158 kb)

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kikunaga, K., Ishikura, K., Terano, C. et al. High incidence of idiopathic nephrotic syndrome in East Asian children: a nationwide survey in Japan (JP-SHINE study). Clin Exp Nephrol 21, 651–657 (2017). https://doi.org/10.1007/s10157-016-1319-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10157-016-1319-z

Keywords

Search

Navigation