Bile Acid Synthesis Defects

What are bile acid synthesis defects?

Bile acids are molecules made in the liver that help break down and digest lipids (fats), eliminate cholesterol and absorb certain vitamins. Bile acid synthesis defects prevent the body from making bile acids and can lead to progressive liver disease.

At The Children's Hospital of Philadelphia, children with bile acid synthesis defects are treated by hepatologists and gastroenterologists in the Biesecker Pediatric Liver Center and the Division of Gastroenterology (GI), Hepatology and Nutrition.