Alagille Syndrome (Alagille-Watson Syndrome)
96
relevant articles (3 outcomes,
6 trials/studies)
found for this Disease
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-Watson Syndrome; Alagille Syndrome 1; Alagille Syndrome 2; Cholestasis with Peripheral Pulmonary Stenosis; Hepatic Ductular Hypoplasia, Syndromatic; Alagille Watson Syndrome; Arteriohepatic Dysplasias; Dysplasias, Arteriohepatic; Syndrome, Alagille; Syndrome, Alagille-Watson; Arteriohepatic Dysplasia; Dysplasia, Arteriohepatic
Relationship Network
Disease Context: Research Results
Related Diseases
Experts
| 1. | Nagasaka, Hironori:
2 articles
(06/2008 - 03/2005)
|
| 2. | Takayanagi, Masaki:
2 articles
(06/2008 - 03/2005)
|
| 3. | Kobayashi, Kunihiko:
2 articles
(06/2008 - 03/2005)
|
| 4. | Chiba, Hitoshi:
2 articles
(06/2008 - 03/2005)
|
| 5. | Yorifuji, Tohru:
2 articles
(06/2008 - 03/2005)
|
| 6. | Larrosa-Haro, Alfredo:
2 articles
(01/2008 - 01/2003)
|
| 7. | Hadchouel, Michelle:
2 articles
(11/2007 - 05/2006)
|
| 8. | Bilgin, N:
2 articles
(08/2001 - 04/2001)
|
| 9. | Haberal, M:
2 articles
(08/2001 - 04/2001)
|
| 10. | Karakayali, H:
2 articles
(08/2001 - 04/2001)
|
Drugs and Biologics
Drugs and Important Biological Agents (IBA) related to Alagille Syndrome:
Therapies and Procedures
| 1. | Liver Transplantation
|
| 2. | Transplants (Transplant)
12/01/2006
- " Two children were weaned from long-term tube feeding after liver transplant because of Alagille syndrome" 10/01/2005
- " From these eleven cases, the indications for liver transplant were acute liver failure (ALF) in seven, biliary atresia in three, and Alagille syndrome in one" 07/01/2002
- " CLINICAL PRESENTATION: A 13-year-old girl with Alagille syndrome presented with oral manifestation of end-stage liver disease that needed liver transplant" 11/01/2002
- " Successful combined orthotopic liver transplant and transcatheter management of atrial septal defect, patent ductus arteriosus, and peripheral pulmonic stenosis in a small infant with Alagille syndrome." 01/01/1990
- " The different diseases of the transplants were: biliary atresia (9), Alagille syndrome (4), deficit alpha 1-antitrypsin (3), autoimmune hepatitis (2), neonatal hepatitis (1), Byler disease (1), Wolman disease (1)"
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| 3. | Transplantation (Transplant Recipients)
07/27/2001
- " METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive" 03/01/2005
- " The main indication for primary transplantation was biliary atresia (10), followed by Byler's disease (2), acute liver failure on cryptogenetic cirrhosis (1), and Alagille syndrome (1)" 04/01/2001
- " It is unusual to see the clinical picture of hepatic failure resulting in cirrhosis and requiring transplantation, but liver transplantation is indicated in Alagille syndrome patients who have chronic cholestasis" 09/01/2002
- " The mean waiting time on the transplantation list was 6.1+/-8.9 months and the main indications for the procedure were biliary atresia, inborn metabolic disorders, liver cirrhosis, liver neoplasms, Alagille syndrome, and fulminant hepatic failure"
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| 4. | Anesthesia
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