Glycogen Storage Disease Type III


Amylo 1,6 Glucosidase Deficiency

Amylo-1,6-Glucosidase Deficiencies

Amylo-1,6-Glucosidase Deficiency

Cori Disease

Cori's Disease

Coris Disease

Debrancher Deficiencies

Debrancher Deficiencies, Glycogen

Debrancher Deficiency

Debrancher Deficiency, Glycogen

Deficiencies, Amylo-1,6-Glucosidase

Deficiencies, Debrancher

Deficiencies, Glycogen Debrancher

Deficiency, Amylo-1,6-Glucosidase

Deficiency, Debrancher

Deficiency, Glycogen Debrancher

Dextrinoses, Limit

Dextrinosis, Limit

Disease, Cori

Disease, Cori's

Disease, Forbes

Forbes Disease

Glycogen Debrancher Deficiencies

Glycogen Debrancher Deficiency

Glycogen Debranching Enzyme Deficiency

Glycogen Storage Disease III

Glycogen Storage Disease Type 3

Glycogenosis 3

Glycogenosis 3s

Limit Dextrinoses

Limit Dextrinosis

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.