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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Alagille syndrome


Other Names for this Disease
  • Alagille-Watson syndrome
  • Arteriohepatic dysplasia
  • Cardiovertebral syndrome
  • Cholestasis with peripheral pulmonary stenosis
  • Hepatic ductular hypoplasia
More Names
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Prognosis


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What is the outlook (prognosis) for individuals with Allagille syndrome?

The outlook for people with Alagille syndrome depends on several factors, including the severity of liver damage and heart problems and the early correction of malabsorption. Predicting who will experience improved bile flow and who will progress to end-stage liver failure is difficult. Fifteen percent of people with Alagille syndrome will eventually require a liver transplant.[1]

Research studies report that 75 percent of children diagnosed with Alagille syndrome live to at least 20 years of age. Because of improvements in liver and heart therapies, this survival rate is increasing. Many adults with Alagille syndrome who improve with treatment lead normal, productive lives. Deaths in people with Alagille syndrome are most often caused by liver failure, heart problems, and blood vessel abnormalities.[1]

Last updated: 11/1/2012

References
  1. Alagille Syndrome. National Digestive Diseases Information Clearinghouse (NDDIC). February 2012; http://digestive.niddk.nih.gov/ddiseases/pubs/alagille/. Accessed 11/1/2012.