《英文msh詞典》Limit Dextrinosis
[同義詞] Limit Dextrinosis
[主題詞] Glycogen Storage Disease Type III
[中文釋義] 局限性糖原貯積病
[英文釋義] An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I,but milder. Massive hepatomegaly,which is present in young children,diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle,liver,and erythrocytes. Six subgroups have been identified,with subgroups Type IIIa and Type IIIb being the most prevalent.