遗传代谢性肝病的病理诊断思路  

Considerations on pathological diagnosis of inherited metabolic liver disease

作　　者：刘晖[1] 梁晨 郑素军[2] 王泰龄[4] Liu Hui;Liang Chen;Zheng Sujun;Wang Tailing(Department of Pathology,Beijing YouAn Hospital,Capital Medical University,Beijing 100069,China;The First Department of Liver Center,Beijing YouAn Hospital,Capital Medical University,Beijing 100069,China;Beijing Municipal Key Laboratory of Liver Failure and Artificial Liver Treatment Research,Beijing YouAn Hospital,Capital Medical University,Beijing 100069,China;Department of Pathology,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京佑安医院病理科,北京100069 [2]首都医科大学附属北京佑安医院肝病中心一科,北京100069 [3]肝衰竭与人工肝治疗研究北京市重点实验室,北京100069 [4]中日友好医院病理科,北京100191

出　　处：《中华肝脏病杂志》2022年第11期1253-1258,共6页Chinese Journal of Hepatology

基　　金：首都卫生发展科研专项重大专项项目(2022-1-2182);北京市医院管理中心重点医学专业发展计划(扬帆计划)(ZYLX202125);北京市大数据精准医疗先进创新中心(1212040205);北京市自然科学基金(7202068)。

摘　　要：肝脏病理活检对遗传代谢性肝病(IMLD)的诊断有重要提示作用。现介绍IMLD的病理诊断思路,并根据肝穿刺组织的形态特点,将IMLD分为5种类型:肝组织形态基本正常型、脂肪变性型、胆汁淤积型、贮积/沉积型及肝炎型,总结了不同损伤模式及常见疾病的的病理特点,以期为正确诊断IMLD提供线索。A liver biopsy has an important suggestive role in the diagnosis of inherited metabolic liver disease(IMLD).This article introduces the IMLD pathological diagnosis considerations,five types of classification of liver biopsy based on the morphological characteristics(basic normal liver tissue morphology,steatosis,cholestatic disease,storage/deposition,and hepatitis),and a summary of the pathological characteristics of different injury patterns and common diseases in order to provide clues for the correct diagnosis.

关 键 词：病理 遗传代谢性肝病 诊断思路 损伤模式 

分 类 号：R575[医药卫生—消化系统]