脾切除治疗小儿肝豆状核变性合并脾功能亢进的疗效分析

目的 探讨脾切除治疗小儿肝豆状核变性合并脾功能亢进的有效性及安全性.方法 回顾分析30例小儿肝豆状核变性合并脾功能亢进而行脾切除的临床资料,对手术前后血细胞和免疫球蛋白的变化进行比较.结果 全部患者术后白细胞及血小板恢复至正常,手术前后免疫球蛋白比较差异无统计学意义.结论 脾切除是小儿肝豆状核变性合并脾功能亢进的主要治疗措施,它能保证患儿继续驱铜治疗.只要充分地进行围手术期处理,脾切除是有效的、可行的、安全的.

Analysis of the therapeutic effect of splenectomy to treat hepatolenticular degeneration complicated with hypersplenism in children

Objective To evaluate the efficacy and safety of spenectomy for the treatment of children with hepatolenticular degeneration combined with hypersplenism. Methods The clinical data of splenectomy for the treatment of children with hepatolenticular degeneration complicated with hypersplenism were analyzed retro- spectively. The comparison was made on the changes of blood cell and pre- and post-opertive immunoglobulin. Results Blood platelet and leucocyte count were increased significantly after operation in all 30 cases. Immunoglobulin pre- and post-operative was no significantly different. Conclusion Splenectomy is valuable in treating children with hepatolenticular degeneration complicated with hypersplenism. It is effective, safe and feasible. It can ensure the copper elimination therapy to continue.