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目的 :初步探讨劈离式肝移植治疗肝豆状核变性病的可行性及治疗效果。方法 :于 2 0 0 4年 9月 15日在西北地区首次实施劈离式原位肝移植治疗一家兄妹 (15岁 ,5岁 ) 2例肝豆状核变性患儿。供肝重量 12 80 g ,行胆道造影后合理设计劈肝路途 ,用超声刀和双极电刀逐步劈离成左半肝 (380 g)和右半肝 (90 0g)。采取“背驮式”移植方法分别将右半肝移给哥哥并置胆总管外引流 ,将左半肝移给妹妹并置胆总管活动性内引流。 结果 :整个手术历时 16h ,顺利完成 2例肝移植 ,除胆红素和转氨酶短暂升高外其他生化指标基本正常。术后 3个月 ,2例患儿生活质量、新肝功能良好 ,哥哥谷草转氨酶 4 0IU/L ,谷丙转氨酶 37IU/L ,总胆红素 2 1μmol/L ,白蛋白 38g/L ;妹妹谷草转氨酶 2 5IU/L ,谷丙转氨酶 17IU/L ,总胆红素 17μmol/L ,白蛋白 4 0 g/L。 结论 :劈离式小儿原位肝移植方法可行 ,对影像和手术技术要求高 ,是有效利用供肝的肝移植技术 ,值得推广。
Objective: To investigate the feasibility and therapeutic effect of split-off liver transplantation in the treatment of Wilson’s disease. METHODS: Two cases of hepatolenticular degeneration in a sibling (15 years old, 5 years old) were treated with split-off orthotopic liver transplantation in the northwest of China on September 15, 2004. The weight of donor liver was 1280 g. After hepatic angiography, the hemihepatic hemisphere was divided into the left hemiliver (380 g) and the right hemiliver (90 0 g). To take the “piggyback” transplantation method, respectively, the right hemilertebra transferred to the brother and common bile duct drainage, the left hemilertebra transferred to the sister and placed bile duct active drainage. Results: The entire operation lasted 16h, 2 cases of liver transplantation were successfully completed, and other biochemical indexes were normal except for the transient increase of bilirubin and transaminase. At 3 months after operation, the quality of life and neohepatic function of the two children were good. Elder asparagine aminotransferase 4 0 IU / L, alanine aminotransferase 37 IU / L, total bilirubin 2 1 μmol / L, albumin 38 g / L, Aminotransferase 2 5IU / L, alanine aminotransferase 17IU / L, total bilirubin 17μmol / L, albumin 40g / L. Conclusion: The method of orthotopic orthotopic liver transplantation in children is feasible and requires high imaging and surgical techniques. It is an effective liver transplantation technique and is worth promoting.