介入治疗8例肝豆状核变性并发严重门静脉高压的疗效

Interventional treatment of severe portal hypertension due to hepatolenticular degeneration:therapeutic evaluation of 8 cases

目的评价介入治疗肝豆状核变性所致门脉高压的疗效。方法分析世纪坛医院介入治疗科8例合并严重门静脉高压的肝豆状核变性患者治疗情况,采用经皮穿刺胃冠状静脉栓塞或部分脾动脉栓塞治疗,比较手术前后血象和门脉压力变化。结果 所有病例均未再出现严重门静脉高压的表现,白细胞于术后1周内明显升高,2周后恢复至正常范围;血小板于术后第2周以后逐渐恢复至正常范围。术后门静脉压力略有升高,但差异无统计学意义。结论介入治疗可缓解肝豆状核变性导致的门静脉高压症,有利于继续恢复驱铜药物治疗。

Objective To evaluate the therapeutic efficacy of sclerotic embolization of esophagogastric varices（SEEV）and partial splenic embolization（PSE）in treating esophagogastric varices and portal hypertension,respectively,in patients with hepatolenticular degeneration.Methods Eight patients with severe portal hypertension complicated with upper gastrointestinal bleeding and hypersplenism were involved in this study.White blood cell（WBC）counts,platelet counts,and portal vein pressure were determined before and after operation,the results were compared with each other.Results No recurrence or complication occurred after operation in all patients.After the treatment（SEEV and PSE）the hepatic function showed no obvious changes.The WBC counts increased obviously in the first week after operation,and returned to normal range in 2 weeks.The platelet counts gradually returned to normal level from the second week.The portal vein pressure after operation went up a little,from（45.13 ± 8.69） cm H2O to（48.63 ± 10.48） cm H2O in SEEV group and to（47.88 ± 11.43） cm H2O in PSE group,but the difference was of no statistic significance（P 〉0.05）.Conclusion Interventional therapy can reduce the portal hypertension caused by hepatolenticular degeneration.The technique is safe and effective,and is very helpful for returning to anti-copper treatment.