摘要
目的复习1例特发性门脉高压(IPH)患者接受肝移植(LT)后第3年出现病情"再发",并进行了相关文献复习,以提高对该病的认识.方法报道1例我们诊治的IPH患者的病例资料,并检索MEDLINE、EMBASE、万方等数据库经LT治疗的IPH患者的研究报道,分析其治疗和转归.结果本文报道的病例为57岁女性,因消化道出血、腹水行LT术,组织病理学检查诊断为IPH;术后随访第3年病情复发,行经皮肝穿刺活检术,病理学检查提示结节性再生性增生(NRH)、轻度汇管区炎症及纤维化,提示IPH再发;文献检索到81例LT治疗的IPH患者,其中42例在LT前诊断为肝硬化;LT后最长随访时间为248个月,8例死亡,其中5例分别在首次LT后3.5月~14年进行肝活检,组织病理学检查提示NRH,3例分别于LT后第7月、第3年和第14年出现具有门脉高压表现的NRH.结论具有严重的门脉高压或肝功能衰竭的IPH患者需要LT治疗.少数患者在LT后可能出现IPH"再发".
Objective The aim of this study was to report a case of patients with idiopathic portal hypertension(IPH)and review the related literatures.Methods One patients with IPH was reported,and the studies on LT for IPH were retrieved from MEDLINE,EMBASE,Wanfang data and other databases.The outcome after LT in patients with IPH was analyzed.Results A 57-year-old female was clinically diagnosed as liver cirrhosis(LC),underwent LT due to gastrointestinal bleeding and ascites,and the final diagnosis was IPH based on the liver histopathology examination;the percutaneous liver biopsy was performed at the third year of follow-up and showed the nodular regenerative hyperplasia(NRH),with mild portal inflammation and fibrosis,suggesting recurrence of IPH;a total of 81 patients with IHP were retrieved from the literatures,and forty-two patients were diagnosed as LC before LT;the longest follow-up period after LT was 248 months and eight patients died;five patients underwent liver biopsy 3.5 month to 14 years after the first LT,and the pathology showed NRH again;among them,3 patients developed NRH with portal hypertension after LT in 7th month,3 years and 14 years,respectively.Conclusions The patients with IPH with severe portal hypertension or liver failure should receive LT,and a few patients might have"recurrence"of IPH,which warrants further investigation.
作者
张维
侯维
刘晖
宋文艳
王莉琳
王征
郑素军
Zhang Wei;Hou Wei;Liu Hui(Liver Disease Center,You’an Hospital,Capital Medical University,Beijing 100069,China)
出处
《实用肝脏病杂志》
CAS
2023年第1期136-139,共4页
Journal of Practical Hepatology
基金
北京市医院管理中心重点医学专业发展计划(扬帆计划)资助项目(编号:ZYLX202125)
北京市肝病研究所/改革与发展-院所合作项目(编号:Y-2020HZ-2)
首都卫生发展科研专项项目(编号:首发2020-1-2181)
关键词
特发性门脉高压
肝移植
预后
复发
Idiopathic portal hypertension
Liver transplantation
Prognosis
Recurrence
