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Journal of the Korean Society of Pediatric Nephrology 2004;8(2): 244-249.
Focal Segmental Glomerulosclerosis in a Child with Prader-Willi Syndrome : A Case of Obesity-associated Focal Segmental Glomerulosclerosis
Hee-Yeon Cho, Dae-Lim Chung, Ju-Hyung Kang, Il-Soo Ha, Hae-Il Cheong, Yong Choi
1Department of Pediatrics, Seoul National Universiry college of Medicine
2Department of Pediatrics, Seoul National Universiry college of Medicine
3Department of Pediatrics, Seoul National Universiry college of Medicine
4Department of Pediatrics, Seoul National Universiry college of Medicine
5Department of Pediatrics, Seoul National Universiry college of Medicine
6Department of Pediatrics, Seoul National Universiry college of Medicine
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ABSTRACT
Obesity-associated focal segmental glomeruloscleropis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features Include obesity, FSGS lesions with glornerulomegaly 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 Pradel-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.
Key words: Prader-Willi syndrome | Focal segmental glomerulosclerosis | Obesity Renal insufficiency
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