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暴发型肝豆状核变性的治疗及预后分析 被引量:9

Prognosis and treatment of fulminant Wilson's disease
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摘要 目的:了解暴发型肝豆状核变性(FWD)的有效治疗方法及影响预后的因素。方法:回顾性分析13例FWD患者。观察采用激素、二巯基丙磺酸钠驱铜、血浆置换联合治疗FWD患者的疗效;对比存活组与死亡组临床特点、生化资料与治疗的差异。结果:13例FWD患者均有重度黄疸,合并重度溶血性贫血7例,自发性腹膜炎5例,肝性脑病8例;凝血酶原活动度(PTA)小于30%者5例。13例患者中采用驱铜联合血浆置换治疗及短期使用激素者7例,存活6例,1例合并自发性腹膜炎且PTA小于30%者死亡;另6例死亡患者中4例合并自发性腹膜炎,4例PTA小于30%。结论:肝衰竭程度及合并感染是决定FWD患者预后的重要因素;采用激素、驱铜、血浆置换是治疗早中期暴发型肝豆状核变性的有效方法之一。 Objective To explore the effective treatment and prognostic factors for fulminant Wilson's disease(FWD).Methods We retrospectively analyzed the clinical characteristics,therapeutic methods and outcomes of 13 FWD patients.We investigated the treatment effect of the joint use of hormones,decoppering,and plasma exchange therapy in patients with FWD,compared the difference in the clinical features,biochemical data and treatment between the survival group and the death group.Results Thirteen patients with FWD presented with acute hepatic failure and severe jaundice: 7 accompanied with severe hemolytic anemia,5 with primary peritonitis,8 with hepatic encephalopathy.Prothrombin activity(PTA) of 5 was below 30% in the 13 patients.Plasma exchange(PE),dimercaptopropansulfonate sodium(DMPS) and short-term methylprednisolone /dexamethasone administration were performed in 7 patients,in which 6 survived and the other 1 who had primary peritonitis with PTA below 30% died.The other 6 patients without above-mentioned treatments all died,in which 4 accompanied with primary peritonitis with PTA all below 30%.Conclusion The level of hepatic failure and the occurrence of infection are the decisive factors for prognosis of patients with FWD.PE with decoppering treatment and corticosteroid administration are effective.
出处 《中南大学学报(医学版)》 CAS CSCD 北大核心 2011年第11期1111-1114,共4页 Journal of Central South University :Medical Science
关键词 暴发型肝豆状核变性 二巯基丙磺酸钠 驱铜 血浆置换 fulminant Wilson's disease dimercaptopropansulfonate sodium decoppering plasma exchange
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