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

Cho, Hee-Yeon; Chung, Dae-Lim; Kang, Ju-Hyung; Ha, Il-Soo; Cheong, Hae-Il; Choi, Yong

J Korean Soc Pediatr Nephrol > Volume 8(2); 2004 > Article

J Korean Soc Pediatr Nephrol 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

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.

Key words: Prader-Willi syndrome | Focal segmental glomerulosclerosis | Obesity | Renal insufficiency