Byler's disease occurs when bile ducts within the liver do not form normally. They become blocked, which causes a build-up of bile in the liver. Byler’s disease is a progressive liver condition (autosomal recessive) initially seen in infants less than 6 months old.
How This Hurts the Liver
Bile is not able to leave the liver through the bile ducts. This causes liver damage. Clinically it includes jaundice, itching, failure to thrive and death from liver cirrhosis.
Some patients with Byler's disease respond to medical therapy. In other children, liver transplant remains standard therapy.