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

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.