Baussan C, Moatti N, Odievre M, Lemonnier A
Pediatrics. 1981 Jan;67(1):107-12.
Investigated were 24 cases of glycogenosis caused by a reduction in liver phosphorylase activity. The intravenous glucagon tolerance test could not discriminate between phosphorylase kinase deficiency [glycogen storage disease (GSD) IX] and phosphorylase deficiency (GSD VI). These two subgroups were distinguished by hemolysate enzyme assays: (1) GSD IX was characterized by a residual phosphorylase kinase activity, a low activation curve for endogenous phosphorylase b and increased amylo-1,6-glucosidase activity. (2) GSD VI was characterized by a normal or increased phosphorylase kinase activity, a slight activation of endogenous phosphorylase b and a normal amylo-1,6-glucosidase activity.
对24例因肝磷酸化酶活性降低引起的糖原贮积病患者进行了研究。静脉注射胰高血糖素耐量试验无法区分磷酸化酶激酶缺乏症（糖原贮积病（GSD）IX型）和磷酸化酶缺乏症（GSD VI型）。通过溶血产物酶分析区分这两个亚组：（1）GSD IX型的特征是残留磷酸化酶激酶活性、内源性磷酸化酶b的激活曲线低以及淀粉-1,6-葡萄糖苷酶活性增加。（2）GSD VI型的特征是磷酸化酶激酶活性正常或增加、内源性磷酸化酶b略有激活以及淀粉-1,6-葡萄糖苷酶活性正常。
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