Cystic fibrosis liver disease occurs as a result of damage and scarring to liver cells which in turn affects the organ’s ability to function. Cystic fibrosis is a recessive genetic condition that clogs the body’s organs (including the pancreas, lungs and liver) with a thick, sticky mucus. This mucus can clog up intra-liver bile ducts and cause long term liver damage. While it is still unclear whether it is a build-up of toxicity within these bile ducts or the obstruction itself which causes most damage, treatments are available. There is, however, currently no definitive cure.
QIMR Berghofer researchers have identified a panel of serum markers (specific blood test indicators) to be used in conjunction with either liver biopsy or ultrasound scans to predict development of serious liver complications, including portal hypertension, in children with cystic fibrosis. They have also identified a mechanism that regulates scar formation in the liver (fibrosis) and are currently assessing potential anti-fibrotic therapies to treat this liver scarring. It is hoped this research will lead to the development of a cure for the condition which 1 in every 2,500 children are born with.
RESEARCH FOCUS
- developing diagnostic tests to assess liver fibrosis in cystic fibrosis patients
- understanding the processes that regulate specific liver cells which produce scar tissue in liver disease with the hope of developing future remedies