改良金氏评分在儿童肝豆状核变性急性肝衰竭肝移植手术时机评估中价值并文献复习

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

目的探讨改良金氏评分在儿童肝豆状核变性急性肝衰竭(WD-ALF)肝移植手术时机评估中的价值。方法回顾性分析2020年1月至2023年5月中山大学附属第三医院收治的4例儿童WD-ALF患者临床资料。其中男2例,女2例;年龄8~15岁。患儿家属均签署知情同意书,符合医学伦理学规定。收集分析患儿病史、一般情况及诊疗过程,使用改良金氏评分对患儿手术时机进行动态评估,评估患儿肝移植手术疗效。检索2000年1月至2023年5月PubMed和中国知网收录的相关文献进行文献复习。英文检索词:Wilson's disease,liver transplantation,pediatric acute liver failure,operation timing等;中文检索词:儿童肝豆状核变性、肝移植、儿童急性肝衰竭、手术时机等。结果患儿入院后完善相关检查后均予内科支持治疗,3例患儿改良金氏评分分别为15、15、11分,分别于入院后3周、5天、3天时行肝移植手术,术后均康复顺利。例4患儿经积极内科治疗后,改良金氏评分从10分逐渐下降至4分,病情缓解出院,暂未接受肝移植治疗。文献复习共检索到9篇相关文献,共10例患儿,其中男1例,女9例;年龄5~17岁,中位年龄13岁。其中有胆红素水平、INR、AST等完整信息的文献8篇共9例患儿,其改良金氏评分为11~16分,中位数14分。大部分评分>11分的患儿接受肝移植手术后预后良好,而2例未接受肝移植治疗的患儿改良金氏评分为14、15分,分别于入院后第10天和第8天死亡。结论肝移植治疗可有效改善WD-ALF患儿预后,改良金氏评分可精准评估患儿病情,把握肝移植治疗的手术时机,评分≥11分的患儿应尽早接受肝移植治疗。

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.