患儿男，9岁，自幼生长缓慢，3岁前多次发生惊厥。来诊时见患儿矮胖，肝大达肋缘下5cm，质中等，空腹血糖为3.0mmol/L，注射肾上腺素后血糖升高甚微。肝活检切片见干细胞内大量PAS阳性物质积聚伴多量脂肪滴。经肝细胞酶检测发现葡萄糖-6-磷酸酶缺陷，当前的治疗原则为 -题库考试答案搜索网

患儿男，9岁，自幼生长缓慢，3岁前多次发生惊厥。来诊时见患儿矮胖，肝大达肋缘下5cm，质中等，空腹血糖为3.0mmol/L，注射肾上腺素后血糖升高甚微。肝活检切片见干细胞内大量PAS阳性物质积聚伴多量脂肪滴。
经肝细胞酶检测发现葡萄糖-6-磷酸酶缺陷，当前的治疗原则为

单选题

2021-07-17 22:02

A、酶替代治疗
B、控制酸中毒
C、进行肝移植
D、首先保持血糖正常并预防和积极治疗感染及控制酸中毒
E、保肝+控制感染和酸中毒

查看答案

正确答案

D

试题解析

标签：医药卫生类中级儿科学（中级）儿科学（中级）模拟卷二

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