Skip to main content

Advertisement

SpringerLink
Log in

Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan

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

Abstract

Background

Congenital nephrotic syndrome is difficult to manage, particularly the Finnish type (CNF), with patients experiencing severe edema, sepsis and thrombosis before kidney transplantation. Further, nephrosis and thrombosis remain problematic after transplantation.

Methods

Of 22 CNF patients managed at our hospital, 14 who underwent kidney transplantation were retrospectively studied. CNF was diagnosed according to standard criteria.

Results

The study population consisted of 3 males and 11 females. Mean gestation period was 36 ± 1.4 weeks and mean birth weight was 2442 ± 454 g (mean placenta to body weight ratio: 0.4). All patients started dialysis at 2.4 ± 1.3 years and underwent kidney transplantation at 5.2 ± 2.0 years. The kidneys were donated by the parents (n = 13), and cadaver (n = 2), including overlap. Mean follow-up period after transplantation was 14.3 ± 8.9 years, and mean age at last observation was 19.5 ± 8.5 years. Two patients had recurrent proteinuria after kidney transplantation; one underwent retransplantation following graft failure and eventually required dialysis, while the second had complete remission after intensive immunosuppressive therapy. There were no cases of thrombosis or serious infections. Mean eGFR at the time of last observation was 57.3 ± 16.5 ml/min/1.73 m2, while mean height SD score was − 2.1 ± 0.9 at the time of transplantation and − 1.5 ± 1.5 at last observation.

Conclusions

Long-term outcome in these 14 CNF patients showed satisfactory graft survival, improved height SD score, and favorable development. Although recurrent proteinuria after transplant was not predictive, it was associated with graft survival rate.

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.

Fig. 1

Similar content being viewed by others

References

  1. Arneil GC. The nephrotic syndrome. Pediatr Clin North Am. 1971;18:547–59.

    Article  PubMed  CAS  Google Scholar 

  2. Ahvenainen EK, Hallman N, Hjelt L. Nephrotic syndrome in newborn and young infants. Ann Paediatr Fenn. 1956;2:227–41.

    Google Scholar 

  3. Holmberg C, Laine J, Ronnholm K, Ala Houhala M, Jalanko H. Congenital nephrotic syndrome. Kidney Int Suppl. 1996;53:S51–6.

    PubMed  CAS  Google Scholar 

  4. Albright SG, Warner AA, Seeds JW, Burton BK. Congenital nephrosis as a cause of elevated alpha-fetoprotein. Obstet Gynecol. 1990;76:969–71.

    PubMed  CAS  Google Scholar 

  5. Mahan JD, Mauer SM, Sibley RK, Vernier RL. Congenital nephrotic syndrome: evolution of medical management and results of renal transplantation. J Pediatr. 1984;105:549–57.

    Article  PubMed  CAS  Google Scholar 

  6. Kestila M, Lenkkeri U, Mannikko M, Lamerdin J, McCready P, Putaala H, et al. Positionally cloned gene for a novel glomerular protein–nephrin–is mutated in congenital nephrotic syndrome. Mol Cell. 1998;1:575–82.

    Article  PubMed  CAS  Google Scholar 

  7. Lenkkeri U, Mannikko M, McCready P, Lamerdin J, Gribouval O, Niaudet PM, et al. Structure of the gene for congenital nephrotic syndrome of the finnish type (NPHS1) and characterization of mutations. Am J Hum Genet. 1999;64:51–61.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  8. Holmberg C, Antikainen M, Ronnholm K, Ala Houhala M, Jalanko H. Management of congenital nephrotic syndrome of the Finnish type. Pediatr Nephrol. 1995;9:87–93.

    Article  PubMed  CAS  Google Scholar 

  9. Papez KE, Smoyer WE. Recent advances in congenital nephrotic syndrome. Curr Opin Pediat. 2004;16:165–70.

    Article  Google Scholar 

  10. Pomeranz A, Wolach B, Bernheim J, Korzets Z, Bernheim J. Successful treatment of Finnish congenital nephrotic syndrome with captopril and indomethacin. J Pediatr. 1995;126:140–2.

    Article  PubMed  CAS  Google Scholar 

  11. Coulthard MG. Management of Finnish congenital nephrotic syndrome by unilateral nephrectomy. Pediatr Nephrol. 1989;3:451–3.

    Article  PubMed  CAS  Google Scholar 

  12. Heaton PA, Smales O, Wong W. Congenital nephrotic syndrome responsive to captopril and indometacin. Arch Dis Child. 1999;81:174–5.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  13. Kovacevic L, Reid CJ, Rigden SP. Management of congenital nephrotic syndrome. Pediatr Nephrol. 2003;18:426–30.

    Article  PubMed  Google Scholar 

  14. Kim MS, Primack W, Harmon WE. Congenital nephrotic syndrome: preemptive bilateral nephrectomy and dialysis before renal transplantation. J Am Soc Nephrol. 1992;3:260–3.

    PubMed  CAS  Google Scholar 

  15. Laine J, Jalanko H, Holthofer H, Krogerus L, Rapola J, von Willebrand E, et al. Post-transplantation nephrosis in congenital nephrotic syndrome of the Finnish type. Kidney Int. 1993;44:867–74.

    Article  PubMed  CAS  Google Scholar 

  16. Patrakka J, Ruotsalainen V, Reponen P, Qvist E, Laine J, Holmberg C, et al. Recurrence of nephrotic syndrome in kidney grafts of patients with congenital nephrotic syndrome of the Finnish type: role of nephrin. Transplantation. 2002;73:394–403.

    Article  PubMed  Google Scholar 

  17. Boute N, Gribouval O, Roselli S, Benessy F, Lee H, Fuchshuber A, et al. NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat Genet. 2000;24:349–54.

    Article  PubMed  CAS  Google Scholar 

  18. Jeanpierre C, Denamur E, Henry I, Cabanis MO, Luce S, Cecille A, et al. Identification of constitutional WT1 mutations, in patients with isolated diffuse mesangial sclerosis, and analysis of genotype/phenotype correlations by use of a computerized mutation database. Am J Hum Genet. 1998;62:824–33.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  19. Sako M, Nakanishi K, Obana M, Yata N, Hoshii S, Takahashi S, et al. Analysis of NPHS1, NPHS2, ACTN4, and WT1 in Japanese patients with congenital nephrotic syndrome. Kidney Int. 2005;67:1248–55.

    Article  PubMed  CAS  Google Scholar 

  20. Uemura O, Nagai T, Ishikura K, Ito S, Hataya H, Gotoh Y, et al. Creatinine-based equation to estimate the glomerular filtration rate in Japanese children and adolescents with chronic kidney disease. Clin Exp Nephrol. 2014;18:626–33.

    Article  PubMed  CAS  Google Scholar 

  21. Matsuo S, Imai E, Horio M, Yasuda Y, Tomita K, Nitta K, et al. Revised equations for estimated GFR from serum creatinine in Japan. Am J Kidney Dis. 2009;53:982–92.

    Article  PubMed  CAS  Google Scholar 

  22. Kim MS, Stablein D, Harmon WE. Renal transplantation in children with congenital nephrotic syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS). Pediatr Transplant. 1998;2:305–8.

    PubMed  CAS  Google Scholar 

  23. Lane PH, Schnaper HW, Vernier RL, Bunchman TE. Steroid-dependent nephrotic syndrome following renal transplantation for congenital nephrotic syndrome. Pediatr Nephrol. 1991;5:300–3.

    Article  PubMed  CAS  Google Scholar 

  24. Flynn JT, Schulman SL, deChadarevian JP, Dunn SP, Kaiser BA, Polinsky MS, et al. Treatment of steroid-resistant post-transplant nephrotic syndrome with cyclophosphamide in a child with congenital nephrotic syndrome. 1992;6:553–5.

  25. Kuusniemi AM, Qvist E, Sun Y, Patrakka J, Ronnholm K, Karikoski R, et al. Plasma exchange and retransplantation in recurrent nephrosis of patients with congenital nephrotic syndrome of the Finnish type (NPHS1). Transplantation. 2007;83:1316–23.

    Article  PubMed  Google Scholar 

  26. Holmberg C, Jalanko H. Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation. Pediatr Nephrol. 2014;29:2309–17.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

The authors would like to thank Dr. Masahiro Ikeda.

Author information

Authors and Affiliations

  1. Department of Pediatric Nephrology, Toho University Faculty of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan

    Yuko Hamasaki & Seiichiro Shishido

  2. Department of Nephrology, Toho University Faculty of Medicine, Tokyo, Japan

    Masaki Muramatsu & Seiichiro Shishido

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

    Yuko Hamasaki, Riku Hamada, Kenji Ishikura, Hiroshi Hataya & Masataka Honda

  4. Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan

    Kenji Ishikura

  5. Department of Urology, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Hiroyuki Satou

  6. Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan

    Koichi Nakanishi

  7. Department of General Pediatrics, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Hiroshi Hataya

Authors
  1. Yuko Hamasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Masaki Muramatsu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Riku Hamada
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kenji Ishikura
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hiroshi Hataya
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Hiroyuki Satou
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Masataka Honda
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Koichi Nakanishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Seiichiro Shishido
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yuko Hamasaki.

Ethics declarations

Conflict of interest

Yuko Hamasaki belongs to a department endowed with sponsorship by Asahi Kasei Pharma Corporation, Novartis Pharma K.K., Chugai Pharmaceutical Co., and Astellas Pharma. The other authors have no potential conflicts of interest to disclose

Ethical approval

The study protocol conformed to the Declaration of Helsinki (2008) and was approved by the Toho University Omori Medical Center institutional ethics committees (approval number M16252) and the IRB of Tokyo Metropolitan Children’s Medical Center (approval number H29b-29).

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hamasaki, Y., Muramatsu, M., Hamada, R. et al. Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan. Clin Exp Nephrol 22, 719–726 (2018). https://doi.org/10.1007/s10157-017-1508-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10157-017-1508-4

Keywords

Search

Navigation