Pediatric Nephrology

, Volume 21, Issue 12, pp 1837–1846

Idiopathic nephrotic syndrome in Polish children - its variants and associations with HLA

Authors

    • Department of Transplantation, Polish-American Institute of PediatricsJagiellonian University
  • Krystyna Sancewicz-Pach
    • Department of Pediatric Nephrology, Polish-American Institute of PediatricsJagiellonian University
  • Anna Moczulska
    • Department of Pediatric Nephrology, Polish-American Institute of PediatricsJagiellonian University
Original Article

DOI: 10.1007/s00467-006-0271-7

Cite this article as:
Krasowska-Kwiecień, A., Sancewicz-Pach, K. & Moczulska, A. Pediatr Nephrol (2006) 21: 1837. doi:10.1007/s00467-006-0271-7

Abstract

HLA-DR and HLA-DQ antigens were investigated in 127 Polish children with idiopathic nephrotic syndrome (INS) followed-up for the median time of 11 years (minimum 7 years). HLA typing was performed using the polymerase chain reaction sequence-specific oligonucleotide probing technique and the microlymphocytotoxicity test. Histopathologic INS categories and a response to therapy were analyzed according to particular HLA associations. The results were compared with 330 healthy individuals. In INS children, we observed an increased frequency of HLA-DR7, DR3/7, DQ2 and DQ8, whereas HLA-DR13, DR15, DQ5 and DQ6 were decreased. In minimal change nephrotic syndrome, a relationship with HLA-DR3, DR7, DR3/7 and DQ2 was found. Evolved from minimal changes, focal segmental glomerulosclerosis was associated with HLA-DR7, while primary focal segmental glomerulosclerosis with HLA-DR4 and DQ8. In steroid-dependence and secondary steroid-resistance, an increased frequency of HLA-DR3, DR7, DR3/7 and DQ2 was documented. In contrast, primary steroid-resistant nephrotic syndrome was associated with HLA-DR4 and DQ8. Steroid-dependent patients bearing HLA-DR3 achieved longer remissions after chlorambucil therapy compared with HLA-DR3-negative. In steroid-resistant focal segmental glomerulosclerosis, a reduced response to cyclosporine A was associated with HLA-DR4. Associations with HLA differentiate between pathoanatomic entities of INS and may influence a response to immunosuppressive therapy.

Keywords

Minimal change nephrotic syndromeFocal segmental glomerulosclerosisSteroid dependenceSteroid resistanceChlorambucilCyclosporineHuman leukocyte antigens

Copyright information

© IPNA 2006