bannerHON
img
HONselect
HONselect
img PATIENT / INDIVIDUAL img MEDICAL PROFESSIONAL img WEB PUBLISHER img
img
 
img
HONcode sites
Khresmoi - new !
HONselect
News
Conferences
Images
  HONselect Search
- English - French - German - Spanish - Portuguese - Italian - Dutch   

  Hierarchy 
- English - French - German - Spanish - Portuguese - Italian - Dutch   

Alagille Syndrome
Definition: 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).

Synonym(s): Arteriohepatic Dysplasia / Dysplasia, Arteriohepatic / Alagille Syndrome 1 / Alagille Syndrome 2 / Alagille-Watson Syndrome / Cholestasis with Peripheral Pulmonary Stenosis / Hepatic Ductular Hypoplasia, Syndromatic / Alagille Watson Syndrome / Arteriohepatic Dysplasias / Dysplasias, Arteriohepatic / Syndrome, Alagille / Syndrome, Alagille-Watson
MeSH 2010 © U.S. National Library of Medicine®.
Browse - New search   

  More information from the Genetics Home Reference Website
What is Alagille syndrome?
Alagille syndrome is a genetic disorder that can affect the liver, heart, and other systems of the body.
Signs and symptoms arising from liver damage in Alagille syndrome may include a yellowish tinge in the skin and the whites of the eyes (jaundice), itching, and deposits of cholesterol in the skin (xanthomas). A liver biopsy may indicate too few bile ducts (bile duct paucity). These ducts carry bile (which helps to digest fats) from the liver to the gallbladder and small intestine.
Heart problems associated with Alagille syndrome may include impaired blood flow ...

How common is Alagille syndrome?
The estimated prevalence of Alagille syndrome is 1 in every 70,000 people. This figure is based on diagnoses of liver disease in infancy, and may be an underestimation due to the varying severity and symptoms of the disorder. The prevalence of this disorder could be as high as 1 in 20,000 people if the frequency of gene mutations that cause Alagille syndrome is considered.


More information from the Genetics Home Reference Website
Browse - New search   

  Web resources for "Alagille Syndrome"
  English (10)  French (1)    Spanish (1)      

= Site with HON description   -   = Site with a robot description
info: enter the site: (click below) domain of the site:  
view the HON description of the site HONcode - Alagille Syndrome - National Digestive Diseases Information Clearinghouse www.digestive.niddk.nih.gov  
view the HON description of the site HONcode - Alagille syndrome - Genetics Home Referenceghr.nlm.nih.gov 4
view the robot description of the site Alagille Syndrome / Family Villagewww.familyvillage.wisc.edu  
view the HON description of the site HONcode - Orphanet: Diseases listwww.orpha.net 4
view the robot description of the site rare-diseases — National Organization for Rare Disorderswww.rarediseases.org  
view the robot description of the site http://www.alagille.org/www.alagille.org 2
view the robot description of the site Whonamedit - Alagille's syndromewww.whonamedit.com  
view the HON description of the site HONcode - Cancer-Prone diseaseswww.atlasgeneticsoncology.org  
view the HON description of the site HONcode - Liver Condionswww.kumc.edu  
view the HON description of the site HONcode - HealthCentral : Health Encyclopedia Index - Awww.healthcentral.com  
 
 

Broader term(s): - Cholestasis, Intrahepatic - Cholestasis - Bile Duct Diseases - Biliary Tract Diseases - Digestive System Diseases - Heart Defects, Congenital - Cardiovascular Diseases - Abnormalities, Multiple - Congenital Abnormalities - Congenital, Hereditary, and Neonatal Diseases and Abnormalities - Genetic Diseases, Inborn

Browse - New search   

  Medical image(s) for "Alagille Syndrome"
No results for this term. Browse other medical images in HONmedia?
Browse - New search   

  Medical News for "Alagille Syndrome"
No results for this term. More health news

Broader term(s): - Congenital Abnormalities

Browse - New search   

  Scientific articles from MEDLINE for "Alagille Syndrome"
  - English - French - German - Spanish - Portuguese - Italian - Dutch   
 All recent articles
 Therapy:  by recall    by precision
 Diagnosis:  by recall    by precision
 Etiology:  by recall    by precision
 Prognosis:  by recall    by precision

Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy

Browse - New search   

  Clinical Trials for "Alagille Syndrome"
No results for this term. Consult others clinical trials from ClinicalTrials.gov
Browse - New search   

  Medical Conferences/Events for "Alagille Syndrome"
No results for this term. Consult HON's world-wide database of medical meetings

Broader term(s): - Cardiovascular Diseases

Browse - New search   

   Refine the search for  "Alagille Syndrome"

Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy


  Broader term(s):
      Diseases
          Congenital, Hereditary, and Neonatal Diseases and Abnormalities
              Genetic Diseases, Inborn 
              Congenital Abnormalities
                  Cardiovascular Abnormalities
                      Heart Defects, Congenital 
                  Abnormalities, Multiple 
      Diseases
          Cardiovascular Diseases
              Cardiovascular Abnormalities
                  Heart Defects, Congenital 
      Diseases
          Digestive System Diseases
              Liver Diseases
                  Cholestasis, Intrahepatic 
              Biliary Tract Diseases
                  Bile Duct Diseases
                      Cholestasis
                          Cholestasis, Intrahepatic 

MeSH 2010 © U.S. National Library of Medicine®.

Browse - New search   



Most frequently used terms List of Rare Disease Add HONselect Extract MeSH
Inicio img Sobre nosotros img Rincón de la prensa img Boletín HON img Mapa del sitio img Política ética img Contactos