Insight on Children's Autoimmune Liver Disease

A team of researchers from the University of Colorado School of Medicine and The Children's Hospital, have made a major breakthrough into the causes of one of the most severe liver diseases in infants - biliary atresia.

This disease is quite rare, as it only affects one in 10,000 newborn children, but it is life-threatening, and before this research, not mush was known about it.

What biliary atresia does is blocking the bile ducts in young infants, preventing bile to flow to the small intestine, and as Cara Mack, MD, leader of the CU research says, “it is fatal if not treated quickly.”

When an infant suffers from biliary atresia, doctors can surgically remove the blocked main bile duct in order to gain some time, but the only treatment is a complicated and expensive liver transplant during infancy or childhood.

As said before, not much was known about this disease, not even what caused it, so CU researchers speculated that the cause could be an infection late in the third trimester of pregnancy or soon after birth.

They explained that the body fights the infection but it doesn't stop after defeating it, and continues to attack the body, and more specifically the bile ducts.

After a research in a mouse model, Mack had two theories over why this is happening: either the bile ducts have been changed and the body's protective system senses it, or it may be that the bile ducts produce a protein similar to those produced by the infection, which launches the body's defenses.

During their investigations, Mack and colleagues found a compound of the immune system called an anti-enolase antibody, which reacts to both virus and bile duct proteins, and that might contribute to the injury of the bile duct in biliary atresia.

This discovery that could lead to new diagnostic tests and why not, a better treatment, was published recently in the journal Gastroenterology.