[Successful treatment of a fulminant-type Wilson's disease with deceased donor liver transplantation after recovery from critical illness].
Nihon Shokakibyo Gakkai Zasshi
; 119(12): 1096-1102, 2022.
Article
in Ja
| MEDLINE
| ID: mdl-36504102
ABSTRACT
A 15-year-old female patient was diagnosed with a fulminant-type Wilson's disease. She had severe illness with a Model for End-Stage Liver Disease score of 25 and new Wilson Index score of 11. She underwent plasma exchanges, hemodiafiltration, and administration of fresh frozen plasma on consecutive days. Finally, she had recovered from severe illness and was discharged from the hospital. After 18 months of waiting time, she underwent deceased liver transplantation and returned to normal daily life. In Japan, the critical shortage of donated organs requires a long waiting time. Previous studies demonstrated that artificial liver support systems, including plasma exchange and hemodiafiltration, could be useful for a fulminant-type Wilson's disease. For such a disease, multidisciplinary bridging treatments are crucial for a successful liver transplantation.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Liver Transplantation
/
End Stage Liver Disease
/
Hepatolenticular Degeneration
Type of study:
Prognostic_studies
Limits:
Adolescent
/
Female
/
Humans
Language:
Ja
Journal:
Nihon Shokakibyo Gakkai Zasshi
Year:
2022
Document type:
Article