title: Two different anesthetic managements of a patient with von Gierke's disease.
abstract: Von Gierke's disease, a form of glycogen storage disturbance, is a rare metabolic disorder with important implications for anesthesiologists. It is caused by the lack of the glucose-6-phosphatase, which is necessary for the liver to convert glycogen to glucose. To avoid severe hypoglycemia, it is crucial to keep oral feeding at intervals 2-3 hr for maintaining a normal blood sugar level. The metabolic derangements of von Gierke's disease may result in serious complications in patients undergoing surgery and anesthesia. We report the anesthetic managements of a patient with von Gierke's disease in two operations with different encounters.
authors.affiliation: Department of Anesthesiology, Far Eastern Memorial Hospital, Pan-Chiao, Taipei, Taiwan, ROC.
authors.forename: I-Ren
authors.initials: IR
authors.lastname: Huang
authors.forename: Wei-Horng
authors.initials: WH
authors.lastname: Jean
authors.forename: Cheng-Wei
authors.initials: CW
authors.lastname: Lu
authors.forename: Chia-Chan
authors.initials: CC
authors.lastname: Wu
authors.forename: Tzu-Yu
authors.initials: TY
authors.lastname: Lin
authors.forename: Yueh-Hsun
authors.initials: YH
authors.lastname: Chuang
authors.forename: Wei-Zen
authors.initials: WZ
authors.lastname: Sun
mesh_terms: D000328:Adult; D000758:Anesthesia / Q000379:methods*; D001786:Blood Glucose / Q000032:analysis; D005953:Glycogen Storage Disease Type I / Q000378:metabolism*; D006801:Humans; D008297:Male
chemical_list: D001786:Blood Glucose