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目的探讨遗传性疾病肝豆状核变性合并严重脾功能亢进患者的有效治疗方法。方法对 5 4例肝豆状核变性合并脾亢而行脾切除的患者进行分析 ,比较手术前后外周血细胞变化。结果全部患者术后白细胞及血小板显著增高 ,而神经症状基本未加重 ,患者能继续驱铜治疗。结论脾切除疗效满意 ,是肝豆状核变性合并严重脾功能亢进的重要辅助治疗措施。
Objective To investigate the effective treatment of Hepatolenticular degeneration combined with severe hypersplenism in hereditary diseases. Methods Fifty-four patients with hepatolenticular degeneration combined with hypersplenism underwent splenectomy were analyzed. The changes of peripheral blood cells before and after operation were compared. Results All patients were significantly higher white blood cells and platelets, and neurological symptoms were not aggravated, the patient can continue to drive copper treatment. Conclusions Satisfactory efficacy of splenectomy is an important adjuvant treatment for hepatolenticular degeneration combined with severe hypersplenism.