Advertisement

Clinical and Experimental Nephrology

, Volume 23, Issue 5, pp 721–722 | Cite as

Two different clinical aspects of Japanese children with asymptomatic nephrotic syndrome at onset

  • Shuichiro FujinagaEmail author
  • Yusuke Gonda
Letter to the editor
  • 76 Downloads

To the Editor,

In clinical practice, it is important to distinguish genetic steroid-resistant nephrotic syndrome (SRNS) from primary SRNS at an early stage of the disease, because genetic SRNS patients may have to receive unnecessary and potentially harmful treatments until diagnosis. Furthermore, although renal prognosis of genetic SRNS is unfavorable, several studies have shown that the risk of post-transplant recurrence in patients with genetic SRNS is very low compared with those with primary SRNS. In this regard, recent articles have revealed that genetic SRNS adults with focal segmental glomerulosclerosis (FSGS) have relatively mild clinical manifestations without a full-blown NS at onset as compared to primary SRNS patients [1, 2]. In contrast, Hiraoka et al. [3] reported favorable clinical courses in asymptomatic NS children at onset who maintained long-term remission after steroid therapy. Therefore, the prognosis of asymptomatic NS patients at onset remains unclear.

Here,...

Notes

Acknowledgements

The authors wish to thank Dr Koichi Nakanishi (Department of Pediatrics, Wakayama Medical University) for performing sequence analysis of the WT1 gene in the patient and her parents.

Compliance with ethical standards

Conflict of interest

The authors declare no conflicts of interest.

Human and animal rights

This article does not contain any studies with human participants and we utilized the data described in our previous study [4]. IRB approval is not applicable for an opinion-based letter.

References

  1. 1.
    Sethi S, Zand L, Nasr SH, Glassock RJ, Fervenza FC. Focal and segmental glomerulosclerosis: clinical and kidney biopsy correlations. Clin Kidney J. 2014;7:531–37.CrossRefGoogle Scholar
  2. 2.
    Sethi S, Glassock RJ, Fervenza FC. Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist. Nephrol Dial Transplant. 2015;30:375–84.CrossRefGoogle Scholar
  3. 3.
    Hiraoka M, Takeda N, Tsukahara H, Kimura K, Takagi K, Hayashi S, Kato E, Ohta K, Sudo M. Favorable course of steroid-responsive nephrotic children with mild initial attack. Kidney Int. 1995;47:1392–3.CrossRefGoogle Scholar
  4. 4.
    Gonda Y, Nishino T, Tomii Y, Sakuraya K, Fujinaga S. Clinical course of asymptomatic nephrotic children without edema. Nihon Shoni Jinzobyo Gakkashi. 2018;31:151–4 (in Japanese).CrossRefGoogle Scholar
  5. 5.
    Lipska BS, Iatropoulos P, Maranta R, Caridi G, Ozaltin F, Anarat A, Balat A, Gellermann J, Trautmann A, Erdogan O, Saeed B, Emre S, Bogdanovic R, Azocar M, Balasz-Chmielewska I, Benetti E, Caliskan S, Mir S, Melk A, Ertan P, Baskin E, Jardim H, Davitaia T, Wasilewska A, Drozdz D, Szczepanska M, Jankauskiene A, Higuita LM, Ardissino G, Ozkaya O, Kuzma-Mroczkowska E, Soylemezoglu O, Ranchin B, Medynska A, Tkaczyk M, Peco-Antic A, Akil I, Jarmolinski T, Firszt-Adamczyk A, Dusek J, Simonetti GD, Gok F, Gheissari A, Emma F, Krmar RT, Fischbach M, Printza N, Simkova E, Mele C, Ghiggeri GM, Schaefer F. PodoNet consortium. Genetic screening in adolescents with steroid-resistant nephrotic syndrome. Kidney Int. 2013;84:206–13.CrossRefGoogle Scholar
  6. 6.
    Maruyama K, Iijima K, Ikeda M, Kitamura A, Tsukaguchi H, Yoshiya K, Hoshii S, Wada N, Uemura O, Satomura K, Honda M, Yoshikawa N. NPHS2 mutations in sporadic steroid-resistant nephrotic syndrome in Japanese children. Pediatr Nephrol. 2003;18:412–6.Google Scholar
  7. 7.
    Cho HY, Lee JH, Choi HJ, Lee BH, Ha IS, Choi Y, Cheong HI. WT1 and NPHS2 mutations in Korean children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2008;23:63–70.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  1. 1.Division of NephrologySaitama Children’s Medical CenterSaitamaJapan
  2. 2.Department of PediatricsJuntendo UniversityTokyoJapan

Personalised recommendations