Mitochondrial Hepatopathies

Mitochondrial hepatopathies are a group of liver diseases related to problems with mitochondria. Mitochondria are the parts of cells responsible for generating the cell’s energy supply. Liver disease can occur if the mitochondria do not work properly.

There are two main types of mitochondrial hepatopathies: fatty acid oxidation defects and respiratory chain disorders. In fatty acid oxidation defects, the mitochondria cannot turn fats into energy. In respiratory chain disorders, the mitochondria do not make enough energy.

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