Diseases

Glycogen Storage Disease Type III

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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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Classification

Broader headings

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See Also

MeSH Record

Synonyms

31 entry terms
  • 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
  • Amylo 1,6 Glucosidase Deficiency
  • Amylo-1,6-Glucosidase Deficiencies
  • Coris Disease
  • Debrancher Deficiencies
  • Debrancher Deficiencies, Glycogen
  • Debrancher Deficiency, Glycogen
  • Deficiencies, Amylo-1,6-Glucosidase
  • Deficiencies, Debrancher
  • Deficiencies, Glycogen Debrancher
  • Deficiency, Amylo-1,6-Glucosidase
  • Deficiency, Glycogen Debrancher
  • Dextrinoses, Limit
  • Dextrinosis, Limit
  • Disease, Cori
  • Disease, Cori's
  • Disease, Forbes
  • Glycogen Debrancher Deficiencies
  • Glycogenosis 3s
  • Limit Dextrinoses

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

Indexing Annotation

do not use /congen & do not coord with INFANT, NEWBORN, DISEASES

MeSH Record

History Note

1991(1989); use GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 3 use GLYCOGENOSIS 1975-1988

MeSH Record

Previous Indexing

  • Glucosidases/metabolism (1966-1974)
  • Glycogenosis (1966-1974)
  • Liver Diseases (1966-1974)

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.565w.202w.449w.520w
  2. C18.452w.648w.202w.449w.520w

AMA Style

References

  1. National Library of Medicine. Glycogen Storage Disease Type III. Medical Subject Headings (MeSH). 2026. Unique ID D006010. http://id.nlm.nih.gov/mesh/2026/D006010
  2. Glycogen Storage Disease Type III. In: Wikipedia. https://en.wikipedia.org/wiki/Glycogen_storage_disease_type_III
  3. Glycogen Storage Disease Type III. In: Wikidata. https://www.wikidata.org/wiki/Q494922