论文部分内容阅读
We report a 13- year-old girl who presented with hepatic failure and hemolytic anemia. Laboratory findings showed a normal serum copper level (104μ g/dl), high urinary copper level (2370μ g/dl), and low serum ceruloplasmin level (14.3μ g/dl). Slit-lamp examination revealed Kays-er-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 (< 1mg/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.
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 Kays-er-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 improved. Management of the copper level can reverse severe liver fibrosis in Wilson’s disease.