Two different clinical aspects of Japanese children with asymptomatic nephrotic syndrome at onset
- 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.  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.
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 . IRB approval is not applicable for an opinion-based letter.
- 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.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