Cystic fibrosis liver disease
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 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. In addition, the iron storage protein ferritin, has also been found to play a key role in enhancing the expression of key genes associated with the development of liver fibrosis. 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.

Our research focuses on:
  • Developing diagnostic tests to assess liver fibrosis in haemochromatosis and 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
Research groups involved in cystic fibrosis liver disease research: