KMID : 0361120010150020240
|
|
Korean Journal of Transplantation 2001 Volume.15 No. 2 p.240 ~ p.245
|
|
Kidney Transplantation Due to Familial Focal Segmental Glomerulosclerosis in 4 Children with Identical HLA-A24 from 2 Families
|
|
½ÅÀçÀÏ/Jae Il Shin
¹é½Â¿¬/ÀÌ¿µ¸ñ/±èÁöÈ«/±èº´±æ/±è¼øÀÏ*/±èÀ¯¼±*/¹Ú±âÀÏ*/È«¼ø¿ø¢Ó/Á¤ÇöÁÖ¢Ó/Seoung Yon Baek/Young Mock Lee/Ji Hong Kim/Pyung Kil Kim/Soon Il Kim*/Yu Seun Kim*/Ki Il Park*/Soon Won Hong¢Ó/Hyeon Joo Jeong¢Ó
|
|
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.
|
|
KEYWORD
|
|
|
|
FullTexts / Linksout information
|
|
|
|
Listed journal information
|
|
|