Case report of hepatic adenoma with hemorrhage in glycogen storage disease type Ⅰa
- VernacularTitle:糖原累积病Ⅰa型继发肝腺瘤出血1例报道
- Author:
Yan LIU
;
Yuheng YUAN
;
Mingsheng MA
;
Wei WANG
;
Zhengqing QIU
- Keywords:
glycogen storage disease type Ⅰa;
hepatic adenoma;
hemorrhage
- From:
Basic & Clinical Medicine
2017;37(2):230-233
- CountryChina
- Language:Chinese
-
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 .
