What's the treatment for alagille syndrome?
Treatment of Alagille syndrome is based on increasing the flow of bile from the liver, maintaining normal growth and development, and preventing or correcting any of the specific nutritional deficiencies that often develop. Because bile flow from the liver to the intestine is slow in Alagille syndrome, medications designed to increase the flow of bile are frequently
prescribed. This may decrease the damage in the liver and improve the digestion of fat in foods that are eaten. Also, itching caused by the buildup of bile in the blood and skin may be relieved by medications including diphenhydramine (Benadryl) and hydroxyzine hydrochloride (Atarax).
Elevations in blood cholesterol also respond to the medications used to increase bile flow. Lowering blood cholesterol usually causes the cholesterol skin deposits to improve. Although reduced flow of bile into the intestine leads to poor digestion of dietary fat, a specific type of fat can still be well digested and therefore infant formulas containing high levels of medium-chain triglycerides - MCT - are usually substituted. Deficiencies of the vitamins A, D, E, and K can usually be corrected by oral doses. Sometimes, surgery is necessary during infancy to help establish the diagnosis of Alagille syndrome by direct examination of the bile duct system.
Alagille syndrome patients often have difficulty absorbing the calories they eat. To prevent malnutrition and growth failure, a high-calorie diet with a lot of protein may be recommended. A feeding tube that delivers large quantities of nutrients overnight may be used.
A small percentage of patients will develop cirrhosis (severe damage to the liver). Liver transplantation is the only option for these patients. A liver transplant also may be the last resort for patients with severe itching, portal hypertension (high blood pressure) and severe growth failure that is not responding to medication. Liver transplantation has been used successfully in these cases. |
