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Published on: July 23, 2020
Two different anesthetic managements of a patient with von Gierke's disease
I-Ren Huang1, Wei-Horng Jean, Cheng-Wei Lu
1Department of Anesthesiology, Far Eastern Memorial Hospital, Pan-Chiao, Taipei, Taiwan, ROC.
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.
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