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糖原累积病Ⅰa型继发肝腺瘤出血1例报道

刘妍 袁裕衡 马明圣 王薇 邱正庆

基础医学与临床2017,Vol.37Issue(2):230-233,4.
基础医学与临床2017,Vol.37Issue(2):230-233,4.

糖原累积病Ⅰa型继发肝腺瘤出血1例报道

Case report of hepatic adenoma with hemorrhage in glycogen storage disease type Ⅰa

刘妍 1袁裕衡 1马明圣 1王薇 1邱正庆1

作者信息

  • 1. 中国医学科学院北京协和医学院北京协和医院儿科,北京100730
  • 折叠

摘要

Abstract

Objective To analyze and summarize the clinical characteristics of patients with spontaneous hemor -rhage of hepatic adenoma in glycogen storage disease type Ⅰa.Methods Reporting 1 case in our hospital and making a summary about general situation , category, etiology, diagnosis and treatment of the hemorrhage of hepatic adenoma with glycogen storage disease type Ⅰa through checking literatures .Results The patient was a 27 year old male who had been diagnosed as glycogen storage disease for 14 years, as well as was first found hepatic adeno-ma at the age of 17 .He once was diagnosed as intra-adenoma bleeding with persistent abdominal pain and dizziness and was underwent selective hepatic artery embolization at the age of 22.Hepatic adenoma in glycogen storage dis-ease typeⅠa generally appeared at the age of puberty .One common complication of this disease was hemorrhage of hepatic adenoma , which can be found by ultrasonography and CT .Clinical management includs observation , selec-tive hepatic artery embolization , radiofrequency ablation , surgical resection and liver transplantation .Conclusions Glycogen storage disease type Ⅰa is an autosomal recessive genetic disease with hepatic adenoma as a common complication of GSD Ⅰa, serious liver adenoma's hemorrhage can be life threatening , the radiological examination can be helpful to detect hepatic adenoma .Then appropriate intervention can improve the life quality and prognosis .

关键词

糖原累积病Ⅰa型/肝腺瘤/出血

Key words

glycogen storage disease type Ⅰa/hepatic adenoma/hemorrhage

分类

医药卫生

引用本文复制引用

刘妍,袁裕衡,马明圣,王薇,邱正庆..糖原累积病Ⅰa型继发肝腺瘤出血1例报道[J].基础医学与临床,2017,37(2):230-233,4.

基金项目

“十二五”国家科技支撑计划 ()

基础医学与临床

OACSCDCSTPCD

1001-6325

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