目的　探讨青霉胺治疗肝豆状核变性的最佳方案。方法　选择青霉胺小剂量组 [5 10mg/ (kg·d) ]、中剂量组 [1116mg/ (kg·d) ]两种剂量治疗肝豆状核变性 ,观察其临床症状、每周日均尿排铜量 (连续观察 8周 )及副作用。结果　治疗前 6周中剂量组较小剂量组驱铜效果好 (P <0 0 1) ,6周后两组驱铜量趋于相对稳定 (P >0 0 5 )。治疗期间两组的副作用无明显差异。结论　肝豆状核变性治疗于急性期 (前 6周 )宜选用中剂量 ,急性期后 (6周后 )宜选用小剂量 Objective To investigate the best regimen of penicillamine in the treatment of Wilson’s disease. Methods Two doses of penicillamine low dose group [5 10 mg / (kg · d)] and middle dose group [1116 mg / (kg · d)] were used to treat Wilson’s disease. The clinical symptoms, The amount of copper (continuous observation of 8 weeks) and side effects. Results Compared with the control group, the copper removal efficiency of middle-dose group was lower than that of the low-dose group (P <0.01). The copper-driving rate tended to be relatively stable after 6 weeks (P> 0.05). There was no significant difference in side effects between the two groups during treatment. Conclusions The treatment of hepatolenticular degeneration in the acute phase (6 weeks before) should be used in a moderate dose, after the acute phase (6 weeks) should choose a small dose