Two different anesthetic managements of a patient with von Gierke's disease.
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.
Huang
I-Ren
IR
Department of Anesthesiology, Far Eastern Memorial Hospital, Pan-Chiao, Taipei, Taiwan, ROC.
Jean
Wei-Horng
WH
Lu
Cheng-Wei
CW
Wu
Chia-Chan
CC
Lin
Tzu-Yu
TY
Chuang
Yueh-Hsun
YH
Sun
Wei-Zen
WZ
D000328:Adult; D000758:Anesthesia / Q000379:methods*; D001786:Blood Glucose / Q000032:analysis; D005953:Glycogen Storage Disease Type I / Q000378:metabolism*; D006801:Humans; D008297:Male
D001786:Blood Glucose