MeSH Browser

MeSH Heading: Glycogen Storage Disease Type IV
Tree Number(s): C16.320.565.202.449.540; C18.452.648.202.449.540
Unique ID: D006011
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D006011
Annotation: do not confuse with ANDERSEN SYNDROME, a potassium-sensitive familial periodic paralysis
Scope Note: An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.
Entry Version: GLYCOGEN STORAGE DIS IV
Entry Term(s): Amylopectinosis; Andersen Disease; Andersen's Disease; Brancher Deficiency; Deficiency, Brancher; Gbe1 Deficiency; Glycogen Branching Enzyme Deficiency; Glycogen Storage Disease Type 4; Glycogenosis 4; Glycogenosis IV; Type IV Glycogenosis
Previous Indexing: Glucosyltransferases/metabolism (1966-1974); Glycogenosis (1966-1974); Liver Diseases (1966-1974)
Public MeSH Note: 1991; see GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 4 see GLYCOGENOSIS 1975-1988
History Note: 1991(1989); use GLYCOGEN STORAGE DISEASE 1989-1990; for GLYCOGENOSIS 4 use GLYCOGENOSIS 1975-1988
Date Established: 1991/01/01
Date of Entry: 1974/12/12
Revision Date: 2012/07/03

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Metabolism, Inborn Errors [C16.320.565]
    - Carbohydrate Metabolism, Inborn Errors [C16.320.565.202]
      - Glycogen Storage Disease [C16.320.565.202.449]
        
        Glycogen Storage Disease Type I [C16.320.565.202.449.448]
        
        Glycogen Storage Disease Type II [C16.320.565.202.449.500]
        
        Glycogen Storage Disease Type IIb [C16.320.565.202.449.510]
        
        Glycogen Storage Disease Type III [C16.320.565.202.449.520]
        
        Glycogen Storage Disease Type IV [C16.320.565.202.449.540]
        
        Glycogen Storage Disease Type V [C16.320.565.202.449.560]
        
        Glycogen Storage Disease Type VI [C16.320.565.202.449.580]
        
        Glycogen Storage Disease Type VII [C16.320.565.202.449.600]
        
        Glycogen Storage Disease Type VIII [C16.320.565.202.449.620]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Metabolism, Inborn Errors [C18.452.648]
    - Carbohydrate Metabolism, Inborn Errors [C18.452.648.202]
      - Glycogen Storage Disease [C18.452.648.202.449]
        
        Glycogen Storage Disease Type I [C18.452.648.202.449.448]
        
        Glycogen Storage Disease Type II [C18.452.648.202.449.500]
        
        Glycogen Storage Disease Type IIb [C18.452.648.202.449.510]
        
        Glycogen Storage Disease Type III [C18.452.648.202.449.520]
        
        Glycogen Storage Disease Type IV [C18.452.648.202.449.540]
        
        Glycogen Storage Disease Type V [C18.452.648.202.449.560]
        
        Glycogen Storage Disease Type VI [C18.452.648.202.449.580]
        
        Glycogen Storage Disease Type VII [C18.452.648.202.449.600]
        
        Glycogen Storage Disease Type VIII [C18.452.648.202.449.620]

Glycogen Storage Disease Type IV Preferred

Concept UI: M0009472
Scope Note: An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.
Terms: Glycogen Storage Disease Type IV Preferred Term
Term UI T018278
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1975); Andersen Disease
Term UI T018274
Date06/15/1984
LexicalTag EPO
ThesaurusID; Andersen's Disease
Term UI T018275
Date02/06/1975
LexicalTag EPO
ThesaurusID UNK (19XX); Brancher Deficiency
Term UI T018277
Date05/31/1988
LexicalTag NON
ThesaurusID; Deficiency, Brancher
Term UI T018276
Date05/31/1988
LexicalTag NON
ThesaurusID UNK (19XX); Gbe1 Deficiency
Term UI T811579
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Amylopectinosis
Term UI T018273
Date05/31/1988
LexicalTag NON
ThesaurusID; Glycogen Storage Disease Type 4
Term UI T750084
Date04/27/2009
LexicalTag NON
ThesaurusID; Glycogenosis 4
Term UI T018279
Date05/31/1988
LexicalTag NON
ThesaurusID; Glycogenosis IV
Term UI T816836
Date01/31/2012
LexicalTag NON
ThesaurusID OMIM (2013); Type IV Glycogenosis
Term UI T608267
Date09/07/2004
LexicalTag NON
ThesaurusID NLM (2006); Glycogen Branching Enzyme Deficiency
Term UI T750964
Date05/12/2009
LexicalTag NON
ThesaurusID

Glycogen Storage Disease Type IV MeSH Descriptor Data 2024