Prognosis and treatment of fulminant Wilson's disease.
10.3969/j.issn.1672-7347.2011.11.014
- Author:
Yi TIAN
1
;
Guozhong GONG
;
Xu YANG
Author Information
1. Center for Liver Disease Research, Second Xiangya Hospital, Central South University, Changsha 410011, China. doctortianyi@yahoo.com.cn
- Publication Type:Journal Article
- MeSH:
Chelating Agents;
therapeutic use;
Combined Modality Therapy;
Copper;
blood;
Female;
Hepatolenticular Degeneration;
therapy;
Humans;
Male;
Methylprednisolone;
therapeutic use;
Plasma Exchange;
methods;
Prognosis;
Retrospective Studies;
Unithiol;
therapeutic use
- From:
Journal of Central South University(Medical Sciences)
2011;36(11):1111-1114
- CountryChina
- Language:Chinese
-
Abstract:
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.
