Focal Segmental Glomerulosclerosis in a Child with Prader-Willi Syndrome: A Case of Obesity-associated Focal Segmental Glomerulosclerosis.
- Author:
Hee Yeon CHO
1
;
Dae Lim CHUNG
;
Ju Hyung KANG
;
Il Soo HA
;
Hae Il CHEONG
;
Yong CHOI
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. ilsooha@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Prader-Willi syndrome;
Focal segmental glomerulosclerosis;
Obesity;
Renal insufficiency
- MeSH:
Biopsy;
Child*;
Edema;
Female;
Glomerulosclerosis, Focal Segmental*;
Humans;
Hypertrophy;
Hypoalbuminemia;
Obesity;
Prader-Willi Syndrome*;
Proteinuria;
Renal Insufficiency
- From:Journal of the Korean Society of Pediatric Nephrology
2004;8(2):244-249
- CountryRepublic of Korea
- Language:English
-
Abstract:
Obesity-associated focal segmental glomerulosclerosis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features include obesity, FSGS lesions with glomerulomegaly and, nephrotic-range proteinuria without edema. A 14- year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Prader-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the possible disease entities that can lead to renal insufficiency through obesity.
