全垂体功能减退合并肝硬化3例及文献综述  

Cirrhosis associated with panhypopituitarism in hypopituitary patients:three cases and literature review

作　　者：袁仙仙 陈适 阳洪波 王林杰 潘慧 朱惠娟 YUAN Xianxian;CHEN Shi;YANG Hongbo;WANG Linjie;PAN Hui;ZHU Huijuan(Key Laboratory of Endocrinology of National Health Commission,Department of Endocrinology,Peking Union Medical College Hospital,Translation Medicine Centre,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100730,China;Chinese Center for Behavioral Medical Research in Growth and Development,Jining 272029,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院内分泌科国家卫生健康委员会内分泌重点实验室协和转化医学中心,北京100730 [2]中国生长发育行为医学研究中心,济宁272029

出　　处：《济宁医学院学报》2018年第2期92-96,101,共6页Journal of Jining Medical University

基　　金：国家临床重点专科建设项目(WBYZ2011-873);中国医学科学院医学与健康科技创新工程项目(CAMS-2016-I2M-1-002)

摘　　要：目的分析全垂体功能减退合并肝硬化患者的临床特点,探讨垂体功能减退在非酒精性脂肪肝病(nonalcoholic fatty liver disease,NAFLD)发病中可能的作用。方法对北京协和医院内分泌科收治的3例全垂体功能减退合并肝硬化患者的临床资料进行回顾性分析,并通过万方数据库、中国知网及Pub Med数据库检索2017年7月前的中英文文献。结果北京协和医院3例全垂体功能减退合并肝硬化患者,年龄25~29岁,均未接受规律激素替代治疗,肝脏活检证实为肝硬化1例,依据临床表现明确诊断2例。生化检查均有血象、凝血功能及肝功能异常,规律激素替代治疗后肝功能有不同程度的改善。文献检索到17篇英文文献,其中病例报道10篇,论著4篇。文献报道的垂体功能减退患者中NAFLD的患病率(2.4%~77.3%)显著高于普通人群。10篇病例报告中11例患者平均(24.4±9.5)岁,其中垂体前叶功能减退5例,全垂体功能减退6例,大部分未接受激素替代治疗,诊断为肝硬化7例(64%)。结论全垂体功能减退患者更易发生NAFLD,且易进展为肝硬化,对于全垂体功能减退患者需重视规律激素替代治疗。Objective To analyze the clinical characteristics of three cases of panhypopituitary patients with cirrhosis.Methods We retrospectively reviewed the medical records of three patients in PUMCH who were diagnosed as panhypopituitarism complicated with cirrhosis.A systematic literature review was performed and the characteristic of nonalcoholic fatty liver disease with hypopituitarism in hypopituitary patients were summarized.Results None of the three patients in PUMCH received regular hormone replacement,two of whom were clinically diagnosed as cirrhosis and another one was cytologically confirmed as cirrhosis.After undergoing regular hormone replacement,there was some improvement observed in the hepatic function of patients in PUMCH.By literature review,we found 17 English articles on hypopituitarism complicated by NAFLD,including 10 case reports,4 original articles and 1 review article.There is increasing prevalence of NAFLD in hypopituitary patients especially for whom the regular hormone replacement was not received.Conclusion Within the patients of panhypopituitarism,NAFLD is particularly severe and more easily progresses to NASH or even cirrhosis.Regular hormone replacement should be emphasized and administrated as early as possible.

关 键 词：垂体功能减退 非酒精性脂肪肝 肝硬化 

分 类 号：R584[医药卫生—内分泌]