两种剂量青霉胺治疗肝豆状核变性的临床观察

The study of two kinds of doses of penicillamine in treating hepatolenticuar degeneration

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 )

Objective To determine the best plan with penicillamine in treating the patients with hepatolenticular degeneration.Methods Use two kinds of doses of penicillamine to treat two groups of the patients with hepatolenticular degeneration,one group using small dose[5~10mg/(kg·d)]and the other moderate dose[11~16mg/(kg·d)].Observe the patients' clinical features,determine the average amount of daily urine copper of every week(total 8 weeks)and then draw the curve of urine copper and side effects.Results (1)The effect of copper driving of the moderate dose was better than that of the small dose during the first six weeks of treatment(P<001).(2)After six weeks,the effects of the two doses were relatively stable(P>005).(3)The difference of the side effects between the two groups was not clear.Conclusion The study suggests that we had better use moderate dose to treat patients with hepatolenticular degeneration in acute stage(the first six weeks) and small dose after acute stage(after six weeks).