| Descriptor English: | Glycogen Storage Disease Type III | ||||||
| Descriptor Spanish: |
Enfermedad del Almacenamiento de Glucógeno Tipo III
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| Descriptor Portuguese: | Doença de Depósito de Glicogênio Tipo III | ||||||
| Descriptor French: | Glycogénose de type III | ||||||
| Entry term(s): |
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 |
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| Tree number(s): |
C16.320.565.202.449.520 C18.452.648.202.449.520 |
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| RDF Unique Identifier: | https://id.nlm.nih.gov/mesh/D006010 | ||||||
| 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. |
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| Annotation: | do not use /congen & do not coord with INFANT, NEWBORN, DISEASES |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| Previous Indexing: |
Glucosidases/metabolism (1966-1974) Glycogenosis (1966-1974) Liver Diseases (1966-1974) |
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| Public MeSH Note: | 1991; see GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 3 see GLYCOGENOSIS 1975-1988 |
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| History Note: | 1991(1989); use GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 3 use GLYCOGENOSIS 1975-1988 |
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| Entry Version: | GLYCOGEN STORAGE DIS III |
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| DeCS ID: | 24393 | ||||||
| Unique ID: | D006010 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 1991/01/01 | ||||||
| Date of Entry: | 1974/12/12 | ||||||
| Revision Date: | 2013/07/08 |
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Glycogen Storage Disease Type III
- Preferred
| Concept UI |
M0009471 |
| 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. |
| Preferred term | Glycogen Storage Disease Type III |
| Entry term(s) |
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 |
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