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Alagille Syndrome

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).
Also Known As:
Alagille Syndrome 1; Alagille Syndrome 2; Alagille Watson Syndrome; Cardiovertebral Syndrome; Cholestasis with Peripheral Pulmonary Stenosis; Hepatic Ductular Hypoplasia, Syndromatic; Hepatofacioneurocardiovertebral Syndrome; Watson Alagille Syndrome; Watson Miller Syndrome; Watson-Miller syndrome; Alagille Watson Syndromes; Arteriohepatic Dysplasias; Cardiovertebral Syndromes; Dysplasias, Arteriohepatic; Hepatofacioneurocardiovertebral Syndromes; Syndrome, Alagille; Syndrome, Alagille Watson; Syndrome, Cardiovertebral; Syndrome, Hepatofacioneurocardiovertebral; Syndrome, Watson Alagille; Syndrome, Watson Miller; Syndromes, Alagille Watson; Syndromes, Cardiovertebral; Syndromes, Hepatofacioneurocardiovertebral; syndrome, Watson-Miller; Arteriohepatic Dysplasia; Dysplasia, Arteriohepatic
Networked: 178 relevant articles (3 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cholestasis
2. Liver Diseases (Liver Disease)
3. Intrahepatic Cholestasis
4. Hypercholesterolemia
5. Gallstones (Gallstone)

Experts

1. Hadchouel, Michelle: 7 articles (11/2007 - 09/2002)
2. Kamath, Binita M: 4 articles (08/2012 - 10/2002)
3. Spinner, Nancy B: 4 articles (06/2012 - 10/2002)
4. Meunier-Rotival, Michèle: 4 articles (11/2007 - 01/2003)
5. Arnon, Ronen: 3 articles (12/2014 - 08/2010)
6. Whitington, Peter F: 3 articles (12/2014 - 06/2002)
7. Kerkar, Nanda: 3 articles (12/2014 - 08/2010)
8. Piccoli, David A: 3 articles (02/2012 - 10/2002)
9. Driancourt, Catherine: 3 articles (11/2007 - 05/2005)
10. Hadchouel, M: 3 articles (02/2001 - 09/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Alagille Syndrome:
1. VitaminsIBA
2. terthienyl (alpha-T)IBA
3. progressive familial intrahepatic 1 CholestasisIBA
4. CholesterolIBA
5. Cholestyramine Resin (Questran)FDA LinkGeneric
6. ApolipoproteinsIBA
7. Hepatic ductular hypoplasiaIBA
8. Lipoproteins (Lipoprotein)IBA
9. LecithinsIBA
10. Neprilysin (Neutral Endopeptidase)IBA

Therapies and Procedures

1. Liver Transplantation
2. Transplants (Transplant)
3. Transplantation (Transplant Recipients)
4. Anesthesia
5. Total Parenteral Nutrition