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Alagille Syndrome (Alagille-Watson 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-Watson Syndrome; Alagille Syndrome 1; Alagille Syndrome 2; Alagille Watson Syndrome; Alagille's Syndrome; Arteriohepatic Dysplasia (AHD); Cardiovertebral Syndrome; Cholestasis with Peripheral Pulmonary Stenosis; Hepatic Ductular Hypoplasia; Hepatic Ductular Hypoplasia, Syndromatic; Hepatofacioneurocardiovertebral Syndrome; Paucity of Interlobular Bile Ducts; Watson Alagille Syndrome; Watson Miller Syndrome; Watson-Miller syndrome; Alagilles Syndrome; Ductular Hypoplasia, Hepatic; Dysplasia, Arteriohepatic (AHD); Hypoplasia, Hepatic Ductular; Syndrome, Alagille; Syndrome, Alagille Watson; Syndrome, Alagille's; Syndrome, Alagille-Watson; Syndrome, Cardiovertebral; Syndrome, Hepatofacioneurocardiovertebral; Syndrome, Watson Alagille; Syndrome, Watson Miller; syndrome, Watson-Miller; Arteriohepatic Dysplasia; Dysplasia, Arteriohepatic
Networked: 303 relevant articles (9 outcomes, 22 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cholestasis
2. Liver Diseases (Liver Disease)
3. progressive familial intrahepatic 1 Cholestasis
4. Pruritus (Itching)
5. Intrahepatic Cholestasis

Experts

1. Loomes, Kathleen M: 13 articles (01/2022 - 02/2007)
2. Kamath, Binita M: 11 articles (01/2022 - 10/2002)
3. Spinner, Nancy B: 9 articles (09/2017 - 10/2002)
4. Rosenthal, Philip: 8 articles (01/2022 - 12/2014)
5. Shneider, Benjamin L: 8 articles (01/2022 - 08/2012)
6. Sokol, Ronald J: 8 articles (01/2022 - 05/2011)
7. Karpen, Saul J: 6 articles (01/2022 - 12/2014)
8. Magee, John C: 6 articles (01/2022 - 12/2014)
9. Molleston, Jean P: 6 articles (01/2022 - 10/2018)
10. Piccoli, David A: 6 articles (10/2016 - 10/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Alagille Syndrome:
1. Bile Acids and Salts (Bile Acids)IBA
2. maralixibatIBA
3. VitaminsIBA
4. Ursodeoxycholic Acid (Urso)FDA LinkGeneric
5. gamma-Glutamyltransferase (gamma-Glutamyl Transpeptidase)IBA
6. Rifampin (Rifampicin)FDA LinkGeneric
7. Member 11 Subfamily B ATP Binding Cassette TransporterIBA
8. LipidsIBA
9. CholesterolIBA
10. bile acid binding proteinsIBA

Therapies and Procedures

1. Therapeutics
2. Liver Transplantation
3. Transplantation
4. Hepatic Portoenterostomy
5. Anesthesia