Clinical Analysis Of 50 Cases Of Glycogen Storage Disease Type Ⅰa With Hepatocellular Adenoma

ObjectiveRetrospective analyse of the patients with hepatocellular adenoma of glycogen storage disease type Ⅰa,including the characteristics,the relationship between genotype and phenotype as well as the treatment.Methods1.Collecting the information of 164 patients who were diagnosed with glycogen storage disease type la in Peking Union Medical College Hospital from July 1985 to April 2017,and analyzing the 50 cases with hepatocellular adenomas retrospectively.2.Gathering the main clinical information of patients,including sex,the onset time of hepatocellular adenoma,the prognosis of adenoma and the treatment.Results1.There were 50 cases of glycogen storage disease type la appearing hepatocellular adenomas and the incidence was 30.5%.Meanwhile,the incidence in the 10+ years old was 69.1%.2.The average age of onset of hepatocellular adenoma was 16.4 ± 4.8 years(5-30 years),mainly in multiple cases(62%),mostly distributed in the left lobes.3.The severe complications of hepatocellular adenoma included hemorrhage(3 cases)and malignant transformation(2 cases).4.Hepatocellular adenoma could lead to or harden anemia.5.The treatment of hepatocellular adenoma in GSDIa patients mainly included observation,transcatheter arterial chemoembolization/transcatheter arterial embolization,radiofrequency ablation and surgical r-esection.6.GSDIa with hepatocellular adenoma had no associations with gender and genotype.Conclusions1.The incidence of hepatocellular adenoma in glycogen storage disease type Ⅰa was 30.5%.Meanwhile,the incidence in the 10+ years old was 69.1%.There was no difference between males and females.2.Multiple adenomas were more common.Most adenomas distributed in the right lobe.3.The most common complication of hepatocellular adenoma was haemorrhage,following malignant transformation.4.No association of genotype with HCA in GSDIa was found in this study.5.Radiofrequency ablation followed by transcatheter arterial chemoembolization could be used as a treatment when the diameter of adenoma was<3 cm.Resection was optional when the diameter of adenoma was ≥5cm.Transcatheter arterial chemoembolization was optional for all above conditions.But it may be ineffective when the diameter of adenoma was>5cm.If hemorrhage occurs,transcatheter arterial chemoembolization would be the first choice.For malignant transformation,surgical resection and transcatheter arterial chemoembolization could both be accepted.6.All patients with GSDIa should be taken long-term follow-up and regularly monitoring of abdominal ultrasound and(or)abdominal CT.Once being diagnosed,patients with GSDIa should be monitored at least once a year.To patients over 10+ years old,they should be monitored for at least once or twice a year according to the occurrence of adenomas.To improve the prognosis,early intervention was recommended.