title: Idiopathic nephrotic syndrome in Polish children - its variants and associations with HLA.
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.
authors.affiliation: Department of Transplantation, Polish-American Institute of Pediatrics, Jagiellonian University, 265 Wielicka St., 30-663 Cracow, Poland. alkk@mp.pl
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Krasowska-Kwiecień
authors.forename: Krystyna
authors.initials: K
authors.lastname: Sancewicz-Pach
authors.forename: Anna
authors.initials: A
authors.lastname: Moczulska
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D020022:Genetic Predisposition to Disease; D006683:HLA-DQ Antigens / Q000235:genetics*; D006684:HLA-DR Antigens / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000235:genetics* / Q000503:physiopathology
chemical_list: D006683:HLA-DQ Antigens; D006684:HLA-DR Antigens