Von Gierke's disease病人的兩種麻醉處置方式

Two Different Anesthetic Managements of A Patient with Von Gierke's Disease

Glycogen storage disease是一種罕見的葡萄糖代謝疾病，對禁食及麻醉有潛藏的危險性。Von Gierke's disease傑耳凱氏病（glycogen storage disease, type la）是由於缺乏glucose-6-phophatase這種酵素，導致肝臟無法將肝糖轉變為葡萄糖。為了避免嚴重的低血糖，每2～3小時進食，以維持適當的血糖濃度是非常重要的。傑耳凱氏病所引起的相關代謝問題，對於麻醉與手術而言，有相當的重要性。我們報告一例患有傑耳凱氏病的26歲男性病人要進行內視鏡尿道切開術的麻醉處置。

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 2-3hr 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.