title: Gastric cancer following a liver transplantation for glycogen storage disease type Ia (von Gierke disease): A case report.
abstract: Glycogen storage disease type Ia (GSD-Ia; also termed von Gierke disease) is an inherited metabolic disorder resulting from a glucose-6-phosphatase deficiency. Liver transplantation is considered to be the most effective treatment for GSD-Ia patients. In the present study, the case of a patient with GSD-Ia who received a liver transplantation at 17 years of age is presented. During the 12 years following transplantation, the patient's quality of life markedly improved. However, recently, the patient was diagnosed withde novogastric cancer following a biopsy. Thus, a total gastrectomy with lymph node dissection was performed and the tumor was histologically determined to be a poorly differentiated adenocarcinoma (histopathological stage, pT4N1M0). The patient recovered well and was discharged on postoperative day 10 without any complications. To the best of our knowledge, this is the first case ofde novogastric cancer in a patient with GSD-Ia to be reported.
authors.affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.
authors.forename: Hua
authors.initials: H
authors.lastname: Xiao
authors.affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.
authors.forename: Jianmin
authors.initials: J
authors.lastname: Bian
authors.affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.
authors.forename: Zhaoming
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of General Surgery, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu 210006, P.R. China.
authors.forename: Aixing
authors.initials: A
authors.lastname: Ding
keywords: de novo gastric cancer; glycogen storage disease; liver transplantation