Gene 'clue' to child liver disease

Freya Stratford developed biliary atresia

Scientists may have found a genetic pathway which could help explain why some children develop a potentially fatal liver disease.

Biliary atresia is diagnosed in approximately 80 children in the UK every year.

The progressive degeneration of liver bile ducts can lead to jaundice and weight loss, and eventually extensive scarring of liver tissue.

The majority of children who develop the condition require a liver transplant before the age of 20.

However, the reasons why a few children develop this condition are not fully understood by doctors.

Gene probe

Researchers at Cincinnati Children's Hospital Medical Center took tissue samples from 14 children with the condition, and looked for any similarities in the genes activated in the tissue.

They found that genes controlling inflammatory reactions in liver tissue were activated - suggesting that some of the body's own immune defences were to blame, turning on the wrong target.

Dr Jorge Bezerra, who led the research, said: "This means that infants with biliary atresia may be very prone to drive an inflammatory response against themselves.

"These inflammatory cells see the biliary system as a foreign target."

The more doctors understand about the reaction that drives biliary atresia, the more chance they have of coming up with a way to halt it.

Catherine Arkley, the chief executive of the Children's Liver Disease Foundation, said: "It's certainly an inflammatory disease - this develops in the bile ducts probably from the time of birth.

"This is certainly interesting research. Doctors certainly do not understand what causes this disease.

"It is one of the lethal diseases to very young children."

The research was published in the Lancet medical journal.