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Clinical and Experimental Nephrology

, Volume 22, Issue 3, pp 719–726 | Cite as

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

  • Yuko Hamasaki
  • Masaki Muramatsu
  • Riku Hamada
  • Kenji Ishikura
  • Hiroshi Hataya
  • Hiroyuki Satou
  • Masataka Honda
  • Koichi Nakanishi
  • Seiichiro Shishido
Original article

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.

Keywords

Congenital nephrotic syndrome Kidney transplantation Long-term outcome 

Notes

Acknowledgements

The authors would like to thank Dr. Masahiro Ikeda.

Compliance with ethical standards

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).

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Copyright information

© Japanese Society of Nephrology 2017

Authors and Affiliations

  • Yuko Hamasaki
    • 1
    • 3
  • Masaki Muramatsu
    • 2
  • Riku Hamada
    • 3
  • Kenji Ishikura
    • 3
    • 4
  • Hiroshi Hataya
    • 3
    • 7
  • Hiroyuki Satou
    • 5
  • Masataka Honda
    • 3
  • Koichi Nakanishi
    • 6
  • Seiichiro Shishido
    • 1
    • 2
  1. 1.Department of Pediatric NephrologyToho University Faculty of MedicineTokyoJapan
  2. 2.Department of NephrologyToho University Faculty of MedicineTokyoJapan
  3. 3.Department of NephrologyTokyo Metropolitan Children’s Medical CenterTokyoJapan
  4. 4.Division of Nephrology and RheumatologyNational Center for Child Health and DevelopmentTokyoJapan
  5. 5.Department of UrologyTokyo Metropolitan Children’s Medical CenterTokyoJapan
  6. 6.Department of Child Health and Welfare (Pediatrics), Graduate School of MedicineUniversity of the RyukyusOkinawaJapan
  7. 7.Department of General PediatricsTokyo Metropolitan Children’s Medical CenterTokyoJapan

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