title: Perioperative management of benign hepatic tumors in patients with glycogen storage disease type Ia.
abstract: Glycogen storage disease type Ia (GSD-Ia; von Gierke disease) is an inherited disorder caused by glucose-6-phosphatase deficiency, and there have been some reports of hepatic tumors in patients with this disease. We report two patients with benign hepatic tumors with GSD-Ia. One is a 19-year-old man who underwent segmentectomy 4 for a focal nodular hyperplasia, and the other is a 31-year-old woman who underwent segmentectomies 3, 5, and 6 for hepatic adenomas. Two significant perioperative complications, resulting from the carbohydrate metabolic disorders, hypoglycemia and metabolic acidosis, occurred in both patients. We managed the metabolic complications successfully by administering a sufficient volume of glucose intravenously. Close perioperative monitoring of blood glucose and lactate concentrations is essential in the perioperative management of patients with GSD-Ia. The intravenous administration of glucose, starting with a smaller dose and then increasing the dose, is adequate management for lactic acidosis with or without hypoglycemia during the perioperative period.
authors.affiliation: Department of Surgery, Division of Frontier Medical Science, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8551, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Oshita
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Itamoto
authors.forename: Hironobu
authors.initials: H
authors.lastname: Amano
authors.forename: Hideki
authors.initials: H
authors.lastname: Ohdan
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Tashiro
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Asahara
mesh_terms: D000138:Acidosis / Q000209:etiology / Q000628:therapy; D018248:Adenoma, Liver Cell / Q000150:complications; D000328:Adult; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000150:complications; D044882:Glucose Metabolism Disorders / Q000209:etiology / Q000628:therapy; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006498:Hepatectomy / Q000009:adverse effects*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000628:therapy; D008107:Liver Diseases / Q000150:complications / Q000601:surgery*; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery; D008297:Male; D019990:Perioperative Care