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目的 探讨肝豆状核变性合并严重脾功能亢进病人的治疗方法 ,分析脾切除在肝豆状核变性病人中的适应证和禁忌证。方法 对 16例肝豆状核变性合并脾亢而行脾切除的病人进行分析和随访观察。结果 全部病人术后白细胞及血小板恢复至正常 ,出血症状控制 ,病人能继续驱铜治疗 ,神经系统症状改善 ;2例病人存在副脾并再次出现脾亢症状。结论 脾切除是肝豆状核变性合并严重脾亢的重要辅助治疗措施。
Objective To investigate the treatment of hepatolenticular degeneration combined with severe hypersplenism in patients with spleen resection in hepatolenticular degeneration patients indications and contraindications. Methods 16 cases of hepatolenticular degeneration combined with hypersplenism and splenectomy were analyzed and followed up. Results All patients recovered white blood cells and platelets to normal after operation. Bleeding symptoms were controlled. Patients continued to drive copper therapy and neurological symptoms improved. Two patients had secondary spleen and reappeared symptoms of hypersplenism. Conclusions Splenectomy is an important adjuvant therapy for hepatolenticular degeneration combined with severe hypersplenism.