Treatment and Prognosis for Alagille Syndrome

The treatment is provides to first increase the bile flow from the liver and then to provide nutrient support to enable normal growth and development.

Treatment Options for Alagille Syndrome

Ursodiol (Actigall, Urso) is the only drug approved by the U.S. Food and Drug Administration to increase bile flow.

Other treatment options that are meant for addressing symptoms include the following:


Medications such as cholestyramine (Questran, Prevalite), rifampin (Rifadin), naltrexone (ReVia, Depade), or antihistamines are generally prescribed to treat pruritus. Hydrating the skin for ensuring necessary moisture and trimming the finger nails to prevent damage from scratching are also recommended.

A surgical procedure called Partial external biliary diversion (PEBD) may provide relief from severe itching.
If PEBD does not improve the condition and the lever damages is irreversible , liver tansplantaion may be needed.

Malabsorption and growth problems:

Oral doses of fats and fat soluble vitamins are provided to infants in whom the syndrome results due to malbasorption. If the oral doses are not tolerated then nutriments are fed through a nasal tube or gastrostomy tube( directly through stomach).


The fatty deposits worsen over the first year. However after medication and PEBD they can be totally eliminated.
Liver failure: Liver translpatation is the last recourse available for treating the Alagille syndrome. A group of experts carefully consider the pros and cons, take the patent family into confidence before recommending liver translpantion.
Prognosis for Alagille syndrome

Prognosis for Alagille Syndrome

The prognosis varies with the varying symptoms. 15% People affected by Allegine syndrome need liver tranplantation. Children with Alagille syndrome live up to 20 years with proper treatment and other medical intervention. Adults in whom the disease is diagnosed early and timely treatment provided lead absolutely normal life. The major cause for death is liver damage. Other causes include heart abnormalities and blood vessel damages.

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