Tian Yi, Gong Guozhong, Yang Xu
Center for Liver Disease Research, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2011 Nov;36(11):1111-4. doi: 10.3969/j.issn.1672-7347.2011.11.014.
To explore the effective treatment and prognostic factors for fulminant Wilson's disease (FWD).
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.
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%.
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.
探讨暴发性威尔逊病（FWD）的有效治疗方法及预后因素。
回顾性分析13例FWD患者的临床特征、治疗方法及转归。研究激素、驱铜及血浆置换联合治疗FWD患者的疗效，比较生存组与死亡组临床特点、生化指标及治疗情况的差异。
13例FWD患者均表现为急性肝衰竭及重度黄疸，其中7例伴有重度溶血性贫血，5例伴有原发性腹膜炎，8例伴有肝性脑病。13例患者中5例凝血酶原活动度（PTA）低于30%。7例患者行血浆置换（PE）、二巯丙磺酸钠（DMPS）及短期甲泼尼龙/地塞米松治疗，其中6例存活，另1例原发性腹膜炎伴PTA低于30%者死亡。其余6例未行上述治疗的患者均死亡，其中4例伴有原发性腹膜炎，PTA均低于30%。
肝衰竭程度及感染的发生是FWD患者预后的决定性因素。PE联合驱铜治疗及应用糖皮质激素有效。
