A noninvasive assessment of hepatic fibrosis is desperately needed to advance the care of children with CF significant liver disease and to provide for measurements during clinical trials. That global assessment might serve as both a predictor/descriptor of disease course but also as a critical biomarker for clinical research. FibroScan® measurement of liver stiffness has great potential to fill this void. The underlying hypothesis of this proposal is that elastography in addition to US can improved the prediction of the development of a nodular liver on US and development of portal hypertension over time in children and young adults with CF.
The aim of this study is to determine if transient elastography (TE), when combined with ultrasound (US) pattern characterization can improve the prediction of progression to a nodular pattern on US, and to confirm the feasibility of obtaining TE measurements in children with Cystic Fibrosis (CF). It will prospectively assess whether TE data are associated with conventional laboratory markers of hepatic fibrosis and determine the variability of TE measurements taken at different sites in the same patient.
Clinical Research Coordinator