Abstract
We report a 13-year-old girl who presented with hepatic failure and hemolytic anemia. Laboratory findings showed a normal serum copper level (104 µg/dl), high urinary copper level (2370 µg/dl), and low serum ceruloplasmin level (14.3 µg/dl). Slit-lamp examination revealed Kayser-Fleischer rings on her cornea, and she was diagnosed with Wilson’s disease. Plasma exchange and continuous hemodiafiltration relieved the serious condition, after that laparoscopic examination was performed. Administration of D-penicillamine and restriction of dietary copper (<1 mg/day) were started, leading to a normalized serum alanine amino transferase (ALT) level. After 3 years, she again underwent laparoscopic examination, and the laparoscopic and histological findings of her liver were obviously improved. Management of the copper level can reverse severe liver fibrosis in Wilson’s disease.
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Sakaida, I., Kawaguchi, K., Kimura, T. et al. D-Penicillamine improved laparoscopic and histological findings of the liver in a patient with Wilson’s disease: 3-year follow-up after diagnosis of Coombs-negative hemolytic anemia of Wilson’s disease. J Gastroenterol 40, 646–651 (2005). https://doi.org/10.1007/s00535-005-1600-5
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DOI: https://doi.org/10.1007/s00535-005-1600-5