自1912年Wilson氏以他的名字命名本病以来,文献相继报导肝豆状核变性除有神经系统症状外,肝脏病可能是本病的唯一临床表现(Barnes等,1925),急性或慢性肝功障碍可为50%患儿的早期表现(Solvis等,1971),病程一般在1～6个月之间。作者报导1例平素健康的10岁男性患儿,以急性肝功能衰竭发病,3周死亡,尸检有亚急性肝纤维化。 Since Wilson’s disease was named after him in 1912, the literature reports on Wilson’s disease in addition to neurological symptoms. Liver disease may be the only clinical manifestation of the disease (Barnes et al., 1925), acute or chronic liver disease Work disability can be an early manifestation of 50% of children (Solvis et al., 1971), and the course of disease is generally between 1 and 6 months. The authors report a normal 10-year-old boy with acute onset of liver failure, 3 weeks of death, autopsy subacute liver fibrosis.