Title: Human leukocyte class II antigens in idiopathic nephrotic syndrome in children
- Aleksandra Krasowska-Kwiecień, Krystyna Sancewicz-Pach, Anna Pogan
- Original articles
- Pediatric Review
- Start page:
- Final page:
- HLA, INS, minimal change nephrotic syndrome, glomerulosclerosis, steroid dependency, steroid resistancy
Involvement of an immune mechanism in the pathogenesis ofidiopathic nephrotic syndrome (INS) has been repeatedly reported. A number of studies documented associations between the disease and certain human leukocyte antigens. Aim of the work. The objective ofthe present research was to investigate HLA class IIfrequencies in children with INS from Southeast Poland. We attemptedto determine the differences between histopathologic forms of nephrotic syndrome andthe response to corticosteroids in relation to certain HLA phenotypes. Material and methods. The study involved a group of 121 nephrotic children followed up for at least fouryears. The control group comprised 330 healthy unrelated individuals. HLA class II antigens were typed with the use ofthe microlymphocytotoxicity test and with the method of sequence specific oligonucleotide DNA probes hybridization (PCR-SSOP). Results. The results showed associations between the general incidence ofINS in children and antigens HLA-DR7, HLA-DQ2, HLA-DQ8 andthe combined occurrence ofHLA-DR3 andDR7. Focal segmental glomerulosclerosis differedfrom minimal change nephrotic syndrome in significantly increased frequency ofHLA-DR4 antigen. The pattern ofHLA antigens was similar in primary and secondary (transformed from minimal change) glomerulosclerosis. Frequently relapsing or steroid dependent nephrotic syndrome was associated with HLA-DR3, HLA-DR3/DR7 andHLA-DQ2, while steroid resistant INS with HLA-DR4. We suggest that HLA-DR3 is specifically related to steroid dependent nephrotic syndrome (also in cases of the development of secondary steroid resistancy), in both minimal change INS and glomerulosclerosis. HLA-DR4 when present in primary steroid resistancy, indicates increased risk of focal segmental glomerulosclerosis.