CAJ | 학술논문

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[11～16mg/(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＜0.01)，6周后两组驱铜量趋于相对稳定(P＞0.05)。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。
목적탐토청매알치료간두상핵변성적최가방안。방법선택청매알소제량조[5～10mg/(kg·d)]、중제량조[11～16mg/(kg·d)]량충제량치료간두상핵변성，관찰기림상증상、매주일균뇨배동량(련속관찰8주)급부작용。결과치료전6주중제량조교소제량조구동효과호(P＜0.01)，6주후량조구동량추우상대은정(P＞0.05)。치료기간량조적부작용무명현차이。결론간두상핵변성치료우급성기(전6주)의선용중제량，급성기후(6주후)의선용소제량。
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＜0.01). (2) After six weeks, the effects of the two doses were relatively stable(P＞0.05). (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).