Kidney Transplantation Due to Familial Focal Segmental Glomerulosclerosis in 4 Children with Identical HLA-A24 from 2 Families / 대한이식학회지

ABSTRACT

Focal segmental glomerulosclerosis (FSGS) is a relatively common glomerular disease which is known to be the final pathway of glomerular injuries caused by variable etiologies. There are some renal diseases that are known to have a tendency of familial inheritance such as adult polycystic kidney disease, thin glomerular basement membrane disease, and Alport's syndrome, nephrotic syndrome with many other diseases. Fanconi et al. described the familial occurrence of the nephrotic syndrome first. Since then, a number of other reports have described the cases of nephrotic syndrome within families, though only a handful of families were confirmed as FSGS with histologic evidence. Recently, reports of familial occurrence of FSGS are increasing in number. These patients have been found to be steroid-resistant and unresponsive to immunosuppressive drugs, and most of them progressed to the end stage renal disease. The specific factors leading to glomerular change are not clearly known, but a genetic predisposition has been postulated. A number of reports pointed out the importance of HLA type as a genetic factor related to the pathogenesis of FSGS but the genetic and immunological linkages in FSGS have not been clearly defined yet. We report cases with 4 patients in two unrelated families with HLA-A24 recovered from FSGS after kidney transplantation.