Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

Authors

  • Yonghui Xia University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology
  • Jianhua Mao University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology
  • Xia Jin University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology
  • Wenjing Wang University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology
  • Lizhong Du University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology
  • Aimin Liu University of Hangzhou School of Medicine; The Children's Hospital of Zhejiang; Department of Nephrology

DOI:

https://doi.org/10.1590/clin.v68i5.76838

Abstract

OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis.

Downloads

Download data is not yet available.

Downloads

Published

2013-05-01

Issue

Section

Clinical Sciences

How to Cite

Xia, Y., Mao, J., Jin, X., Wang, W., Du, L., & Liu, A. (2013). Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China. Clinics, 68(5), 628-631. https://doi.org/10.1590/clin.v68i5.76838