Management of children with congenital nephrotic syndrome: challenging treatment paradigms

Dufek S., Holtta T., Trautmann A., Ylinen E., ALPAY H., Ariceta G., ...More

NEPHROLOGY DIALYSIS TRANSPLANTATION, vol.34, no.8, pp.1369-1377, 2019 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 34 Issue: 8
  • Publication Date: 2019
  • Doi Number: 10.1093/ndt/gfy165
  • Journal Name: NEPHROLOGY DIALYSIS TRANSPLANTATION
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.1369-1377
  • Keywords: bilateral nephrectomies, congenital nephrotic syndrome, genotype-phenotype correlation, management approach, NPHS1, DIFFUSE MESANGIAL SCLEROSIS, STEROID-RESISTANT, GENOTYPE/PHENOTYPE CORRELATIONS, GLOMERULAR PROTEIN, 1ST YEAR, MUTATIONS, NPHS1, NEPHRIN, SURVIVAL, LIFE
  • Çukurova University Affiliated: Yes

Abstract

Background. Management of children with congenital nephrotic syndrome (CNS) is challenging. Bilateral nephrectomies followed by dialysis and transplantation are practiced in most centres, but conservative treatment may also be effective.