方基盖氏病 von Gierke＇s disease

互联网2013-11-14

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亦称肝肾性糖原病（hepatorenal glycogeno- sis）。是一种先天性糖原蓄积病。即肝细胞和肾脏曲管上皮细胞内有糖原特殊的积存。此种糖原在代谢上不具活性，不受激素的影响。肝脏的葡萄糖-6-磷酸酶活性甚低。

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