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Chinese Journal of Hepatic Surgery(Electronic Edition) ›› 2023, Vol. 12 ›› Issue (06): 661-668. doi: 10.3877/cma.j.issn.2095-3232.2023.06.014

• Clinical Research • Previous Articles     Next Articles

Value of revised King's score in evaluation of live transplantation timing for children with acute liver failure due to Wilson's disease and literature review

Lei Lyu, Xiao Feng, Kaiming He, Kaining Zeng, Qing Yang, Haijin Lyu, Huimin Yi, Shuhong Yi, Yang Yang, Binsheng Fu()   

  1. Center for Liver Surgery/Organ Transplantation, Lingnan Hospital, the Third Affiliated Hospital of Sun Yat-sen University, Institute of Organ Transplantation of Sun Yat-sen University, Guangdong Organ Transplantation Research Center, Guangzhou 510630, China
  • Received:2023-08-22 Online:2023-12-10 Published:2023-11-23
  • Contact: Binsheng Fu

Abstract:

Objective

To evaluate the value of revised King's score in evaluating the timing of liver transplantation for children with acute liver failure due to Wilson's disease (WD-ALF).

Methods

Clinical data of 4 children with WD-ALF admitted to the Third Affiliated Hospital of Sun Yat-sen University from January 2020 to May 2023 were retrospectively analyzed. Among them, 2 patients were male and 2 female, aged from 8 to 15 years. The informed consents of all patients were obtained and the local ethical committee approval was received. Medical history, baseline data and diagnosis and treatment of these children were collected and analyzed. The timing of surgery was dynamically evaluated by using the revised King's score. Clinical efficacy of liver transplantation was also assessed. Relevant studies and literature review were conducted in PubMed and CNKI from January 2000 to May 2023 using the keywords of Wilson's disease, liver transplantation, pediatric acute liver failure and operation timing in both English and Chinese.

Results

Upon admission, all children received medical support treatments after relevant examinations. The revised King's scores of 3 children were 15, 15 and 11, and underwent liver transplantation respectively at 3 weeks,5 d and 3 d after admission. All patients were recovered smoothly. After active medical treatments, the revised King's score of case 4 was gradually decreased from 10 to 4. The patient was discharged after disease was relieved without undergoing liver transplantation. 9 related studies including 10 children were searched, including 1 male and 9 female, aged from 5 to 17 years, with a median age of 13 years. Complete information, such as bilirubin level, INR and AST, were reported in 8 studies consisting of 9 children. The revised King's score was ranged from 11 to 16, with a median of 14. A majority of children with revised King's score of >11 obtained favorable prognosis after liver transplantation. 2 children with revised King's score of 14 and 15 who did not undergo liver transplantation died on the 10th and 8th day after admission.

Conclusions

Liver transplantation can effectively improve the clinical prognosis of children with WD-ALF. The revised King's score can accurately evaluate the severity of disease and grasp the timing of liver transplantation. Children with a revised King’s score of ≥11 should undergo liver transplantation promptly.

Key words: Liver transplantation, Wilson's disease, Liver failure, Children, Operation timing, The revised King's score

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