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 .%目的：分析和总结糖原累积病Ⅰa型继发肝腺瘤破裂出血患者的临床特征。方法对北京协和医院收治的1例糖原累积病Ⅰa型继发肝腺瘤破裂出血患者临床资料进行报道。结合文献进行分析，对肝腺瘤的概况、分型、病因、诊断及治疗方法做一总结。结果患者为27岁男性，诊断糖原累积病14年，17岁时出现肝腺瘤，22岁时因持续腹痛、头晕就诊当地医院，诊断腺瘤内出血，行选择性肝动脉栓塞治疗。糖原累积病Ⅰa型继发肝腺瘤好发年龄为青春期，肝腺瘤并发症之一为出血，通过超声、CT等影像学检查可协助诊断，治疗方法多种，包括观察、选择性肝动脉栓塞、射频消融、手术切除、肝移植等。结论糖原累积病Ⅰa型是一种常染色体隐性遗传病，严重肝腺瘤破裂出血可危及生命，保留通过影像学监测及早发现肝腺瘤并且适当干预可以提高患者的生活治疗，改善预后。
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