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Liver Disorders and Diseases

Mitochondrial Hepatopathies

What are Mitochondrial Hepatopathies?

Mitochondrial hepatopathies are a special group of liver diseases related to a problem with the way mitochondria are functioning. This leads to damage or poor function of the liver.

There are two main types of mitochondrial hepatopathies. In respiratory chain disorders, the mitochondria don't make enough energy. This is because the tools in the mitochondria did not form completely and are not able to do their job correctly. In fatty acid oxidation defects, the mitochondria are unable to turn fats into energy. Mitochondria need several enzymes (proteins) to change fat into energy. In this type of disorder, the mitochondria are missing some of those enzymes. This means they can't turn the fats we eat into energy.

Treatment

Mitochondrial Hepatopathies are treated in the Pediatric Liver Center at Hopkins Children's.


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