糖元儲積症(glycogen storage disease)或称von Gierke氏病是一种少見的新陈代謝障碍疾病。1929年von Gierke氏曾报告二例由于糖元沉積所引起的肝、腎肥大症,并确定了本病的特殊性,此后欧美各國逐漸有不少零星的病例报告,有些僅是臨床观察,部分并有病理解剖的証实。在國内文献中僅有二例的报告,一例为高、谷二氏(1939)所报告,另一例为 Glycogen storage disease or von Gierke’s disease is a rare metabolic disorder. 1929 von Gierke’s had reported two cases of liver and kidney hypertrophy caused by glycogen deposition, and to determine the particularity of the disease, since then there are many sporadic cases reported in Europe and the United States, some only clinical observation, part of And pathological anatomy confirmed. In the domestic literature, there are only two reports, one of which is reported by Gao and Gu (1939) and the other as