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Journal of the Korean Society of Pediatric Nephrology 2002;6(2): 243-250.
소아기 류마티스 관절염 환아에서 발생한 이차성 신유전분증 1 례
문재훈, 이숙진, 강미선, 정우영
1인제대학교 의과대학 부산 백병원 소아과학 교실
2인제대학교 의과대학 부산 백병원 소아과학 교실
3인제대학교 의과대학 부산 백병원 병리학 교실
4인제대학교 의과대학 부산 백병원 소아과학 교실
A Case of Secondary Renal Amyloidosis in Children with Juvenile Rheumatoid Arthritis
Jae-Hoon Moon, Suk-Jin Lee, Mi-Seon Kang, Woo-Yeong Chung
1Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
2Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
3Department of Pathology, Inje University, College of Medicine, Busan Paik Hospital
4Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
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ABSTRACT
Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain at)d characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.
Key words: secondary renal amyloidosis | juvenile rheumatoid arthritis | children
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