Clinical efficacy of liver transplantation for Wilson's disease
10.3760/cma.j.cn421203-20211125-00296
- VernacularTitle:肝移植治疗肝豆状核变性的临床疗效
- Author:
Xinghua HUANG
1
;
Yi JIANG
;
Lizhi LV
;
Yuyang GUO
;
Xiangyu PENG
;
Huanzhang HU
;
Jianyong LIU
;
Qiucheng CAI
;
Fang YANG
;
Chuan JIANG
Author Information
1. 中国人民解放军联勤保障部队第九〇〇医院肝胆外科 肝移植中心，福州 350025
- Keywords:
Liver transplantation;
Wilson disease;
Neuropsychiatric symptom
- From:
Chinese Journal of Organ Transplantation
2022;43(6):358-363
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical efficacy of liver transplantation for Wilson's disease(WD).Methods:From January 1999 to November 2021, clinical data were retrospectively reviewed for 16 recipients with WD undergoing liver transplantation.There were 9 males and 7 females with an age range of 29.5(14~54)years.They were followed up by telephone, outpatient services and hospitalization.The starting point of follow-up was operation date.And recipient death was an endpoint.Postoperative survival, improvement of neuropsychiatric symptom, changes of corneal K-F ring, altered levels of liver function and serum copper-protein at Month 1 post-operation were observed.The follow-up deadline was November 24, 2021.Results:15 recipients underwent classical orthotopic liver transplantation and the other one recipient underwent living-related liver transplantation.No perioperative deaths occurred.All 16 recipients were followed up for 122(6~260)months.The 1-, 5-, and 10-year survival rates were 93.8%、85.2%and 75.8%, respectively.Among 10 recipients with corneal K-F ring positive with varying degrees after operation and was disappeared in 2 recipients at 7 and 11 months.Among 5 recipients with neuropsychiatric manifestation, 4 recipients showed ameliorative neuropsychic symptoms with varying degrees after operation and 1 recipient died.All the levels of liver function and serum copper-protien of all recipients recovered obviously in 1 month and the 1-, 5-, and 10-year post-operation.Conclusions:Classical orthotopic liver transplantation and living-related liver transplantation not only effectively improves copper metabolism of patient with WD and relieves their severe neurological manifestation, but also improves their life and prolongs survival, which is worthy of clinical promotion.
