Familial steroid-responsive nephrotic syndrome and HLA antigens in Bengalichildren

We investigated the major histocompatibility complex class I and II loci in three Bengali families with nine children affected with steroid-sensitive nephrotic syndrome (SSNS). A sequence-specific primer (SSP) of DNA typing m ethod was used to detect human leukocyte antigens (HLA). The unaffected sib lings and their parents were also studied. Similar to previous reports, the re was a high frequency of HLA-DR7.1 (DRB1*0701), DR53 (DR B4*01011-0104) a nd DQ2 (DQB2*0201-3) antigens in the affected children. However, there was a similar finding in the unaffected children and their parents. HLA-DR7.1 p robably was not a causative factor. since it had no predictive value for th e occurrence or the severity of SSNS in the affected families. Siblings wit h identical HLA typing behaved differently (they either had or did not have SSNS), In these families there was no correlation between predisposition t o the nephrotic syndrome and the genetic determinant responsible for HLA.