A Case of Secondary Renal Amyloidosis in Children with Juvenile Rheumatoid Arthritis

소아기 류마티스 관절염 환아에서 발생한 이차성 신유전분증 1 례

  • Moon Jae-Hoon (Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital) ;
  • Lee Suk-Jin (Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital) ;
  • Kang Mi-Seon (Department of Pathology, Inje University, College of Medicine, Busan Paik Hospital) ;
  • Chung Woo-Yeong (Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital)
  • 문재훈 (인제대학교 의과대학 부산 백병원 소아과학 교실) ;
  • 이숙진 (인제대학교 의과대학 부산 백병원 소아과학 교실) ;
  • 강미선 (인제대학교 의과대학 부산 백병원 병리학 교실) ;
  • 정우영 (인제대학교 의과대학 부산 백병원 소아과학 교실)
  • Published : 2002.10.01

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.

저자들은 소아기 류마티스 관절염으로 진단되어 간헐적으로 NSAID를 투여 받아 오던 중학교 신체검사에서 우연히 발견된 단백뇨와 혈뇨를 주소로 내원하였던 12세 여아에서 신생검상 신장의 아밀로이드의 침착을 확인하여 이차성 유전분증으로 진단된 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords