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Alagille Syndrome Summary

Description: 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 Show All >>

Networked: 178 relevant articles (3 outcomes, 10 trials/studies) for this Disease

Key Drugs and Agents for Alagille Syndrome

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Vitamins : 1 outcome in 1 result : IBA
  2. terthienyl (alpha-T) : 1 outcome in 1 result : IBA
  3. progressive familial intrahepatic 1 Cholestasis : 2 studies in 23 results : IBA
  4. Cholesterol : 2 studies in 9 results : IBA
  5. Cholestyramine Resin (Questran) : 2 studies in 3 results : FDA 18 Generic
  6. Apolipoproteins : 2 studies in 2 results : IBA
  7. Hepatic ductular hypoplasia : 1 study in 24 results : IBA
  8. Lipoproteins (Lipoprotein) : 1 study in 5 results : IBA
  9. Lecithins : 1 study in 2 results : IBA
  10. Neprilysin (Neutral Endopeptidase) : 1 study in 2 results : IBA
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Diseases Related to Alagille Syndrome

  1. Cholestasis
  2. Liver Diseases (Liver Disease)
  3. Intrahepatic Cholestasis
  4. Hypercholesterolemia
  5. Gallstones (Gallstone)
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Key Therapies for Alagille Syndrome

Efficacy Chart >>
  1. Liver Transplantation : 1 outcome 3 studies in 34 results
  2. Transplants (Transplant) : 8 results
  3. Transplantation (Transplant Recipients) : 5 results
  4. Anesthesia : 3 results
  5. Total Parenteral Nutrition : 2 results
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