Prognosis and treatment of fulminant Wilson's disease / 中南大学学报(医学版)
Journal of Central South University(Medical Sciences)
;
(12): 1111-1114, 2011.
Artigo
em Chinês
| WPRIM
| ID: wpr-814467
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Troca Plasmática
/
Prognóstico
/
Terapêutica
/
Unitiol
/
Sangue
/
Metilprednisolona
/
Quelantes
/
Estudos Retrospectivos
/
Terapia Combinada
/
Cobre
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Feminino
/
Humanos
/
Masculino
Idioma:
Chinês
Revista:
Journal of Central South University(Medical Sciences)
Ano de publicação:
2011
Tipo de documento:
Artigo
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