Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Early Onset of Minimal change Nephrotic Syndrome during Treatment with D-penicillamine in a Patient with Wilson's Disease

월슨병 환아에서 D-penicillamine 사용 중 조기 발병한 미세변화형 신증후군 1례

  • Kim Ki-Soo (Departments of Pediatrics, College of Medicine, Konyang Uniuersity) ;
  • Lim Jae-Woo (Departments of Pediatrics, College of Medicine, Konyang Uniuersity) ;
  • Ko Kyong-Og (Departments of Pediatrics, College of Medicine, Konyang Uniuersity) ;
  • Kim Yoon-Mee (Departments of Pathology, College of Medicine, Konyang Uniuersity) ;
  • Cheon Eun-Jung (Departments of Pediatrics, College of Medicine, Konyang Uniuersity)
  • 김기수 (건양대학교 의과대학 소아과학교실) ;
  • 임재우 (건양대학교 의과대학 소아과학교실) ;
  • 고경옥 (건양대학교 의과대학 소아과학교실) ;
  • 김윤미 (건양대학교 의과대학 병리학교실) ;
  • 천은정 (건양대학교 의과대학 소아과학교실)
  • Published : 2004.10.01
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Abstract

Wilson's disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson's disease. However, nephrotoxicity can occur after D-penlcillamlne treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syn,frome in a 3-year-old'girl with Wilson's disease 3 weeks after initiation of D-penicillamine.

월슨병은 구리 대사 이상으로 간, 뇌, 각막, 신 및 건혈구에 구리가 침착되어 생기는 상염색체 열성 유전성 질환이다. D-penicillamine이 주된 치료로서 10% 정도의 환자에서 가역적인 부작용이 발생 할 수 있다 저자는 3세된 여아에서 D-penicillamine치료 3주만에 미세변화형 신증후군이 발생하였고 투약 중단만으로 완전 관해된 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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