Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis Liver Disease

Cystic fibrosis is an inherited condition in which cell secretions are thick and sticky, instead of thin and slippery. The secretions obstruct passageways and ducts, leading to severe internal damage. Patients with cystic fibrosis sometimes develop advanced liver disease (CFLD). Hepatic cirrhosis can occur with CFLD, as a result of the inflammation and scarring caused by clogged bile ducts.

PUSH is a prospective longitudinal study that aims to determine the utility of abdominal ultrasound to predict the development of cirrhosis in patients with cystic fibrosis. Participants will undergo abdominal ultrasound at enrollment and, based on the outcome, will be placed in one of four groups. Within a five year period subjects will undergo other sample collection procedures in addition to abdominal ultrasound. This study will also monitor the effects of cystic fibrosis on associated pulmonary and nutritional issues.