Greene H L, Parker P H, Hoyumpa A M, Hall S D
J Lab Clin Med. 1986 Feb;107(2):118-22.
Patients with deficient activity of hepatic glucose-6-phosphatase (glycogen storage disease type I [GSD-I]) have fasting-induced hypoglycemia, lactic acidemia, hyperuricemia, hyperlipidemia, and a markedly increased capacity for ethanol elimination. The mechanism(s) responsible for the rapid ethanol elimination is not known but has been thought to be directly related to the enzyme defect. We postulated however, that the increased elimination of ethanol was an adaptive phenomenon that would revert toward normal with correction of other blood abnormalities by long-term maintenance of normal blood glucose concentration. Six patients were observed before treatment (group A), and four of the six were observed again 3 to 6 months after dietary treatment had normalized all blood abnormalities (group B). Patients received 16 ml/m2 absolute ethanol as a 5% solution in 0.9% sodium chloride over a 20-minute period. The rate of ethanol elimination was significantly greater (P less than 0.03) in group A than in group B (55.1 +/- 11.1 vs. 37.5 +/- 8.6 mg/dl/hr). Changes in lactate level after ethanol were also significant between the two groups (P less than 0.005). Group A showed a decrease from 9.4 +/- 0.5 to 6.4 +/- 0.4 mEq/L, whereas group B showed an increase in lactate level from 2.7 +/- 0.2 to 4.4 +/- 0.64 mEq/L. Ethanol induced no significant change in blood glucose concentration in group A, whereas there was a significant increase (P less than 0.03) in group B from 93 +/- 6 to 123 +/- 9 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)
肝葡萄糖-6-磷酸酶活性缺乏的患者（I型糖原贮积病[GSD-I]）会出现空腹诱导的低血糖、乳酸性血症、高尿酸血症、高脂血症，以及乙醇清除能力显著增强。导致乙醇快速清除的机制尚不清楚，但一直被认为与酶缺陷直接相关。然而，我们推测，乙醇清除增加是一种适应性现象，通过长期维持正常血糖浓度纠正其他血液异常后，这种现象会恢复正常。观察了6例治疗前的患者（A组），其中4例在饮食治疗使所有血液异常恢复正常3至6个月后再次接受观察（B组）。患者在20分钟内接受16 ml/m2的无水乙醇，以5%的溶液溶于0.9%的氯化钠中。A组的乙醇清除率显著高于B组（P<0.03）（55.1±11.1对37.5±8.6 mg/dl/小时）。两组之间乙醇摄入后乳酸水平的变化也有显著差异（P<0.005）。A组乳酸水平从9.4±0.
