男孩4岁，自幼生长缓慢，多次晨起发生惊厥。来诊时见患儿矮胖，肝肿大达肋缘下6cm,质中等硬；空腹血糖值为2.3mmol/L，注射肾上腺素后血糖升髙甚微。肝活检切片见肝细胞内大量PAS阳性物质积聚伴多量脂肪滴。诊断首先考虑

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