MeSH Browser

MeSH Heading: Mucopolysaccharidosis III
Tree Number(s): C16.320.565.202.715.650; C16.320.565.595.600.650; C17.300.550.575.650; C18.452.648.202.715.650; C18.452.648.595.600.650
Unique ID: D009084
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D009084
Scope Note: Mucopolysaccharidosis characterized by heparitin sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
Entry Version: MPS III
Entry Term(s): Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase Deficiency; Heparan Sulfate Sulfatase Deficiency; MPS 3 A; MPS 3 B; MPS 3 C; MPS 3 D; MPS III A; MPS III B; MPS III C; MPS III D; MPS IIIA; MPS IIIB; MPS IIIC; MPS IIID; MPS3A; MPS3B; MPS3C; Mucopolysaccharidosis 3; Mucopolysaccharidosis Type 3 A; Mucopolysaccharidosis Type 3 A Sanfilippo Syndrome; Mucopolysaccharidosis Type 3 B; Mucopolysaccharidosis Type 3 C; Mucopolysaccharidosis Type 3 D; Mucopolysaccharidosis Type IIIA; Mucopolysaccharidosis Type IIIB; Mucopolysaccharidosis Type IIIC; Mucopolysaccharidosis Type IIID; N-Acetyl-alpha-D-Glucosaminidase Deficiency; N-Acetylglucosamine-6-Sulfatase Deficiency; N-Acetylglucosamine-6-Sulfate Sulfatase Deficiency; NAGLU Deficiency; Polydystrophic Oligophrenia; San Filippo's Syndrome; Sanfilippo Syndrome; Sanfilippo Syndrome A; Sanfilippo Syndrome B; Sanfilippo Syndrome C; Sanfilippo Syndrome D; Sanfilippo's Syndrome; Sulfamidase Deficiency
Previous Indexing: Carbohydrate Metabolism, Inborn Errors (1966-1976); Mental Retardation (1966-1976); Mucopolysaccharides/metabolism (1966-1974); Mucopolysaccharidosis (1974)
Public MeSH Note: 92; was MUCOPOLYSACCHARIDOSIS 3 1991; was see under MUCOPOLYSACCHARIDOSIS 1975-90
Online Note: use MUCOPOLYSACCHARIDOSIS III to search MUCOPOLYSACCHARIDOSIS 3 1975-91
History Note: 92; was MUCOPOLYSACCHARIDOSIS 3 1991; was see under MUCOPOLYSACCHARIDOSIS 1975-90
Date Established: 1991/01/01
Date of Entry: 1974/12/12
Revision Date: 2016/06/28

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]
      - Mucopolysaccharidoses [C16.320.565.202.715]
        
        Mucopolysaccharidosis I [C16.320.565.202.715.640]
        
        Mucopolysaccharidosis II [C16.320.565.202.715.645]
        
        Mucopolysaccharidosis III [C16.320.565.202.715.650]
        
        Mucopolysaccharidosis IV [C16.320.565.202.715.655]
        
        Mucopolysaccharidosis VI [C16.320.565.202.715.670]
        
        Mucopolysaccharidosis VII [C16.320.565.202.715.675]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Metabolism, Inborn Errors [C16.320.565]
    - Lysosomal Storage Diseases [C16.320.565.595]
      - Mucopolysaccharidoses [C16.320.565.595.600]
        
        Mucopolysaccharidosis I [C16.320.565.595.600.640]
        
        Mucopolysaccharidosis II [C16.320.565.595.600.645]
        
        Mucopolysaccharidosis III [C16.320.565.595.600.650]
        
        Mucopolysaccharidosis IV [C16.320.565.595.600.655]
        
        Mucopolysaccharidosis VI [C16.320.565.595.600.670]
        
        Mucopolysaccharidosis VII [C16.320.565.595.600.675]

Skin and Connective Tissue Diseases [C17]
- Connective Tissue Diseases [C17.300]
  - Mucinoses [C17.300.550]
    - Mucopolysaccharidoses [C17.300.550.575]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Metabolism, Inborn Errors [C18.452.648]
    - Carbohydrate Metabolism, Inborn Errors [C18.452.648.202]
      - Mucopolysaccharidoses [C18.452.648.202.715]
        
        Mucopolysaccharidosis I [C18.452.648.202.715.640]
        
        Mucopolysaccharidosis II [C18.452.648.202.715.645]
        
        Mucopolysaccharidosis III [C18.452.648.202.715.650]
        
        Mucopolysaccharidosis IV [C18.452.648.202.715.655]
        
        Mucopolysaccharidosis VI [C18.452.648.202.715.670]
        
        Mucopolysaccharidosis VII [C18.452.648.202.715.675]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Metabolism, Inborn Errors [C18.452.648]
    - Lysosomal Storage Diseases [C18.452.648.595]
      - Mucopolysaccharidoses [C18.452.648.595.600]
        
        Mucopolysaccharidosis I [C18.452.648.595.600.640]
        
        Mucopolysaccharidosis II [C18.452.648.595.600.645]
        
        Mucopolysaccharidosis III [C18.452.648.595.600.650]
        
        Mucopolysaccharidosis IV [C18.452.648.595.600.655]
        
        Mucopolysaccharidosis VI [C18.452.648.595.600.670]
        
        Mucopolysaccharidosis VII [C18.452.648.595.600.675]

Mucopolysaccharidosis III Preferred

Concept UI: M0014168
Scope Note: Mucopolysaccharidosis characterized by heparitin sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
Terms: Mucopolysaccharidosis III Preferred Term
Term UI T027143
Date01/01/1999
LexicalTag NON
ThesaurusID; Sanfilippo Syndrome
Term UI T843588
Date05/03/2013
LexicalTag NON
ThesaurusID GHR (2014); Polydystrophic Oligophrenia
Term UI T027144
Date12/05/1974
LexicalTag NON
ThesaurusID UNK (19XX); Sanfilippo's Syndrome
Term UI T027145
Date12/05/1974
LexicalTag EPO
ThesaurusID UNK (19XX); Mucopolysaccharidosis 3
Term UI T027146
Date09/12/1990
LexicalTag NON
ThesaurusID NLM (1992); San Filippo's Syndrome
Term UI T027147
Date05/24/1990
LexicalTag EPO
ThesaurusID NLM (1991)

MPS III A Narrower

Concept UI: M0014169
Terms: MPS III A Preferred Term
Term UI T027148
Date09/12/1990
LexicalTag NON
ThesaurusID NLM (1992); Mucopolysaccharidosis Type IIIA
Term UI T825078
Date06/27/2012
LexicalTag NON
ThesaurusID OMIM (2013); MPS3A
Term UI T783001
Date12/29/2010
LexicalTag ABB
ThesaurusID; MPS 3 A
Term UI T783002
Date12/29/2010
LexicalTag ABB
ThesaurusID; Mucopolysaccharidosis Type 3 A
Term UI T783003
Date12/29/2010
LexicalTag NON
ThesaurusID; MPS IIIA
Term UI T825074
Date06/27/2012
LexicalTag ABB
ThesaurusID OMIM (2013); Sanfilippo Syndrome A
Term UI T783005
Date12/29/2010
LexicalTag EPO
ThesaurusID; Mucopolysaccharidosis Type 3 A Sanfilippo Syndrome
Term UI T783006
Date12/29/2010
LexicalTag EPO
ThesaurusID; Sulfamidase Deficiency
Term UI T812007
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Heparan Sulfate Sulfatase Deficiency
Term UI T783004
Date12/29/2010
LexicalTag NON
ThesaurusID

MPS III B Narrower

Concept UI: M0014170
Terms: MPS III B Preferred Term
Term UI T027149
Date09/12/1990
LexicalTag NON
ThesaurusID NLM (1992); Mucopolysaccharidosis Type IIIB
Term UI T825079
Date06/27/2012
LexicalTag NON
ThesaurusID OMIM (2013); Mucopolysaccharidosis Type 3 B
Term UI T783007
Date12/29/2010
LexicalTag NON
ThesaurusID; Sanfilippo Syndrome B
Term UI T783009
Date12/29/2010
LexicalTag EPO
ThesaurusID; MPS3B
Term UI T783010
Date12/29/2010
LexicalTag ABB
ThesaurusID; MPS 3 B
Term UI T783011
Date12/29/2010
LexicalTag ABB
ThesaurusID; NAGLU Deficiency
Term UI T783012
Date12/29/2010
LexicalTag ABX
ThesaurusID; MPS IIIB
Term UI T825075
Date06/27/2012
LexicalTag ABB
ThesaurusID OMIM (2013); N-Acetyl-alpha-D-Glucosaminidase Deficiency
Term UI T783023
Date12/29/2010
LexicalTag NON
ThesaurusID

MPS III C Narrower

Concept UI: M0014171
Terms: MPS III C Preferred Term
Term UI T027150
Date09/12/1990
LexicalTag NON
ThesaurusID NLM (1992); Mucopolysaccharidosis Type 3 C
Term UI T783017
Date12/29/2010
LexicalTag NON
ThesaurusID; Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase Deficiency
Term UI T783018
Date12/29/2010
LexicalTag NON
ThesaurusID; Sanfilippo Syndrome C
Term UI T783019
Date12/29/2010
LexicalTag EPO
ThesaurusID; MPS 3 C
Term UI T783020
Date12/29/2010
LexicalTag ABB
ThesaurusID; MPS3C
Term UI T783021
Date12/29/2010
LexicalTag ABB
ThesaurusID; Mucopolysaccharidosis Type IIIC
Term UI T825080
Date06/27/2012
LexicalTag NON
ThesaurusID OMIM (2013); MPS IIIC
Term UI T783022
Date12/29/2010
LexicalTag ABB
ThesaurusID

MPS III D Narrower

Concept UI: M0014172
Terms: MPS III D Preferred Term
Term UI T027151
Date09/12/1990
LexicalTag NON
ThesaurusID NLM (1992); MPS IIID
Term UI T825081
Date06/27/2012
LexicalTag NON
ThesaurusID NLM (2013); Mucopolysaccharidosis Type IIID
Term UI T825082
Date06/27/2012
LexicalTag NON
ThesaurusID OMIM (2013); MPS 3 D
Term UI T783025
Date12/29/2010
LexicalTag ABB
ThesaurusID; Mucopolysaccharidosis Type 3 D
Term UI T783026
Date12/29/2010
LexicalTag NON
ThesaurusID; N-Acetylglucosamine-6-Sulfate Sulfatase Deficiency
Term UI T783027
Date12/29/2010
LexicalTag NON
ThesaurusID; N-Acetylglucosamine-6-Sulfatase Deficiency
Term UI T812014
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Sanfilippo Syndrome D
Term UI T783028
Date12/29/2010
LexicalTag EPO
ThesaurusID

Mucopolysaccharidosis III MeSH Descriptor Data 2025