J Korean Soc Pediatr Nephrol.  2004 Oct;8(2):244-249.

Focal Segmental Glomerulosclerosis in a Child with Prader-Willi Syndrome: A Case of Obesity-associated Focal Segmental Glomerulosclerosis

• Cho HY
• Chung DL
• Kang JH
• Ha IS
• Cheong HI
• Choi Y

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.

Keyword

Prader-Willi syndrome; Focal segmental glomerulosclerosis; Obesity; Renal insufficiency

MeSH Terms

Biopsy
Child*
Edema
Female
Glomerulosclerosis, Focal Segmental*
Humans
Hypertrophy
Hypoalbuminemia
Obesity
Prader-Willi Syndrome*
Proteinuria
Renal Insufficiency