Focal Segmental Glomerulosclerosis in a Child with Prader-Willi Syndrome: A Case of Obesity-associated Focal Segmental Glomerulosclerosis
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초록
비만성 사구체경화증(obesity-associated focal segmental glomerulosclerosis)은 비만과 부종이 없는 신증후군 범위의 단백뇨, 사구체 비대 및 경화 등의 임상상을 보이는 질환으로, 다수의 환자에서 신부전으로 진행되는 것으로 알려져 있다. 연구자들은 부종 없이 심한 단백뇨와 저알부민혈증을 보인 14세의 Prader-Willi 증후군 여아에서 신생검을 통하여 사구체비후와 메산지움 증식이 동반된 국소성 분절성 사구체경화 소견을 관찰하였다. 이로써 소아의 Prader-Willi 증후군에서도 비만성 사구체경화증에 의한 신부전으로의 진행 위험이 있음을 알리는 바이다.
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.
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