Journal List > Korean J Pediatr Gastroenterol Nutr > v.5(2) > 1110435

Korean J Pediatr Gastroenterol Nutr. 2002 Sep;5(2):206-212. Korean.
Published online Sep 30, 2002.
Copyright © 2002 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
A Case of Minimal Change Nephrotic Syndrome Associated with D-penicillamine Therapy of Wilson's Disease
Ki Won Oh, Se Young Kim, Hwan Suk Lee, Byung Ho Choe, Cheol Woo Ko and Ja Hoon Koo
Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.

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.

Keywords: Wilson's disease; D-penicillamine; Side effect; Nephrotic syndrome; Trientine