Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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A Case of Minimal Change Nephrotic Syndrome Associated with D-penicillamine Therapy of Wilson's Disease

윌슨병 환자에서 D-penicillamine 치료 중 발생한 미세변화형 신증후군 1례

  • Oh, Ki-Won (Department of Pediatrics, Kyungpook National University School of Medicine) ;
  • Kim, Se-Young (Department of Pediatrics, Kyungpook National University School of Medicine) ;
  • Lee, Hwan-Suk (Department of Pediatrics, Kyungpook National University School of Medicine) ;
  • Choe, Byung-Ho (Department of Pediatrics, Kyungpook National University School of Medicine) ;
  • Ko, Cheol-Woo (Department of Pediatrics, Kyungpook National University School of Medicine) ;
  • Koo, Ja-Hoon (Department of Pediatrics, Kyungpook National University School of Medicine)
  • 오기원 (경북대학교 의과대학 소아과학교실) ;
  • 김세영 (경북대학교 의과대학 소아과학교실) ;
  • 이환석 (경북대학교 의과대학 소아과학교실) ;
  • 최병호 (경북대학교 의과대학 소아과학교실) ;
  • 고철우 (경북대학교 의과대학 소아과학교실) ;
  • 구자훈 (경북대학교 의과대학 소아과학교실)
  • Received : 2002.08.17
  • Accepted : 2002.08.31
  • Published : 2002.09.30
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Abstract

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

윌슨병의 치료제로 구리 흡착제인 D-penicillamine이 주로 사용되고 있으나, 심각한 부작용이 발생할 경우 투약을 중단하고 trientine 등을 대체약으로 사용할 수 있다. 저자들은 윌슨병으로 D-penicillamine 사용 도중 6개월만에 미세변화형 신증후군이 발생한 후 trientine으로 치료약을 대체 후 관해가 유도된 1례를 경험하였기에 보고하는 바이다.

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