MeSH Browser

MeSH Heading: Alagille Syndrome
Tree Number(s): C06.130.120.135.250.125; C06.552.150.125; C14.240.400.044; C16.131.077.065; C16.131.240.400.044; C16.320.051
Unique ID: D016738
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D016738
Scope Note: A multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS. Alagille syndrome may result from heterogeneous gene mutations, including mutations in JAG1 on CHROMOSOME 20 (Type 1) and NOTCH2 on CHROMOSOME 1 (Type 2).
Entry Term(s): Alagille Syndrome 1; Alagille Syndrome 2; Alagille Watson Syndrome; Alagille's Syndrome; Alagille-Watson Syndrome; Arteriohepatic Dysplasia; Arteriohepatic Dysplasia (AHD); Cardiovertebral Syndrome; Cholestasis with Peripheral Pulmonary Stenosis; Dysplasia, Arteriohepatic; Hepatic Ductular Hypoplasia; Hepatic Ductular Hypoplasia, Syndromatic; Hepatofacioneurocardiovertebral Syndrome; Paucity of Interlobular Bile Ducts; Watson Alagille Syndrome; Watson Miller Syndrome; Watson-Miller syndrome
Previous Indexing: Abnormalities, Multiple (1981-1991); Bile Ducts, Intrahepatic/abnormalities (1980-1991); Cholestasis, Intrahepatic (1979-1991); Pulmonary Artery/abnormalities (1988-1991)
Public MeSH Note: 92
History Note: 92
Date Established: 1992/01/01
Date of Entry: 1991/04/29
Revision Date: 2013/07/08

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)

Digestive System Diseases [C06]
- Biliary Tract Diseases [C06.130]
  - Bile Duct Diseases [C06.130.120]
    - Cholestasis [C06.130.120.135]
      - Cholestasis, Intrahepatic [C06.130.120.135.250]
        
        Alagille Syndrome [C06.130.120.135.250.125]
        
        Liver Cirrhosis, Biliary [C06.130.120.135.250.250]

Digestive System Diseases [C06]
- Liver Diseases [C06.552]
  - Cholestasis, Intrahepatic [C06.552.150]
    - Alagille Syndrome [C06.552.150.125]
    - Liver Cirrhosis, Biliary [C06.552.150.250]

Cardiovascular Diseases [C14]
- Cardiovascular Abnormalities [C14.240]
  - Heart Defects, Congenital [C14.240.400]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Congenital Abnormalities [C16.131]
  - Abnormalities, Multiple [C16.131.077]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Congenital Abnormalities [C16.131]
  - Cardiovascular Abnormalities [C16.131.240]
    - Heart Defects, Congenital [C16.131.240.400]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]

Alagille Syndrome Preferred

Concept UI: M0025493
Scope Note: A multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS. Alagille syndrome may result from heterogeneous gene mutations, including mutations in JAG1 on CHROMOSOME 20 (Type 1) and NOTCH2 on CHROMOSOME 1 (Type 2).
Terms: Alagille Syndrome Preferred Term
Term UI T050177
Date01/01/1999
LexicalTag EPO
ThesaurusID; Arteriohepatic Dysplasia (AHD)
Term UI T844113
Date05/14/2013
LexicalTag NON
ThesaurusID GHR (2014); Alagille Watson Syndrome
Term UI T763950
Date01/12/2010
LexicalTag NON
ThesaurusID; Alagille's Syndrome
Term UI T840848
Date04/18/2013
LexicalTag NON
ThesaurusID GHR (2014); Alagille-Watson Syndrome
Term UI T811985
Date11/15/2011
LexicalTag EPO
ThesaurusID; Arteriohepatic Dysplasia
Term UI T050178
Date01/09/1991
LexicalTag NON
ThesaurusID; Cardiovertebral Syndrome
Term UI T763946
Date01/12/2010
LexicalTag NON
ThesaurusID; Cholestasis with Peripheral Pulmonary Stenosis
Term UI T521200
Date10/03/2002
LexicalTag NON
ThesaurusID; Dysplasia, Arteriohepatic
Term UI T050179
Date01/09/1991
LexicalTag NON
ThesaurusID NLM (1992); Hepatic Ductular Hypoplasia, Syndromatic
Term UI T521201
Date10/03/2002
LexicalTag NON
ThesaurusID; Hepatofacioneurocardiovertebral Syndrome
Term UI T763947
Date01/12/2010
LexicalTag NON
ThesaurusID; Watson Alagille Syndrome
Term UI T770104
Date04/22/2010
LexicalTag EPO
ThesaurusID; Watson-Miller syndrome
Term UI T770105
Date04/22/2010
LexicalTag EPO
ThesaurusID; Watson Miller Syndrome
Term UI T763949
Date01/12/2010
LexicalTag EPO
ThesaurusID

Alagille Syndrome 1 Narrower

Alagille Syndrome 2 Narrower

Paucity of Interlobular Bile Ducts Related

Alagille Syndrome MeSH Descriptor Data 2025