deficiencies. Diet can also be a crucial factor in improving quality of life when living with ALGS. Medication Several medications are used to improve bile flow, including ursodiol (Actigall or Urso). These medications differ in their rates of success. Certain drugs may be used to reduce itching (pruritus), such as cholestyramine and rifampin. While these medications can reduce pruritus, the itching often is reduced when bile flow is improved via ursodiol or liver transplant. Many patients with Alagille syndrome have nutritional and/or malabsorption issues which often hinders normal growth. Patients benefit from vitamin A, D, E, and K supplements because the reduced bile flow makes it difficult to absorb and utilize these vitamins. A high-calorie diet is very important, and often requires a gastrostomy tube to maintain the high caloric intake. Maralixibat (Livmarli) was approved for medical use in the United States in September 2021. Surgery Surgery is common in more severe cases on Alagille syndrome, especially for patients with liver disease or end-stage liver failure. Liver transplants can either be a complete liver transplant from a deceased organ donor, or a partial transplant from a living donor. Liver transplants can be difficult in ALGS patients because heart defects are common along with the liver failure, and such intense surgeries are dangerous for cardiac patients because they cannot handle the stress of surgery and general anesthesia. Partial biliary diversion has been used to significantly reduce pruritus, jaundice, and xanthoma caused by poor bile flow in patients with bile duct paucity. A portion of the bile produced by the liver is directed through a surgically created stoma into a plastic pouch on the patient's lower right abdomen. The pouch is periodically drained as it fills with bile. Patients with biliary atresia may require a Kasai procedure to improve bile drainage; however, later liver transplantation is still often necessary. See also Progressive familial intrahepatic cholestasis References External links GeneReviews/NCBI/UW/NIH entry on Alagille syndrome OMIM entries on Alagille syndrome This article incorporates public domain text from The U.S. National Library of Medicine Accessory digestive gland disorders Ciliopathy Hepatology Rare syndromes Syndromes affecting the hepatobiliary system Congenital disorders of digestive system Cardiogenetic disorders Syndromes affecting the heart Syndromes with tumors Diseases named for discoverer