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Glycogen Storage Disease Type III MeSH Descriptor Data 2022


MeSH Heading
Glycogen Storage Disease Type III
Tree Number(s)
C16.320.565.202.449.520
C18.452.648.202.449.520
Unique ID
D006010
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D006010
Annotation
do not use /‌congen & do not coord with INFANT, NEWBORN, DISEASES
Scope Note
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.
Entry Version
GLYCOGEN STORAGE DIS III
Entry Term(s)
Amylo-1,6-Glucosidase Deficiency
Cori Disease
Cori's Disease
Debrancher Deficiency
Deficiency, Debrancher
Forbes Disease
Glycogen Debrancher Deficiency
Glycogen Debranching Enzyme Deficiency
Glycogen Storage Disease III
Glycogen Storage Disease Type 3
Glycogenosis 3
Limit Dextrinosis
Previous Indexing
Glucosidases/metabolism (1966-1974)
Glycogenosis (1966-1974)
Liver Diseases (1966-1974)
Public MeSH Note
1991; see GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 3 see GLYCOGENOSIS 1975-1988
History Note
1991(1989); use GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 3 use GLYCOGENOSIS 1975-1988
Date Established
1991/01/01
Date of Entry
1974/12/12
Revision Date
2013/07/08
Glycogen Storage Disease Type III Preferred
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