Longitudinal Assessment of Transient Elastography in Cystic Fibrosis

Noninvasive monitoring of liver fibrosis is an unmet critical need in cystic fibrosis (CF) liver disease. Liver biopsy is infrequently used in CF due to the patchy nature of liver involvement and is not suitable for longitudinal studies of progressive liver disease in CF. Indeed, unlike many other liver diseases, synthetic dysfunction is rare in CF liver disease. Portal hypertension due to hepatic fibrosis mediates most if not all of the complications seen in CF liver disease. The Longitudinal Assessment of Transient Elastography in Cystic Fibrosis (ELASTIC-CF) study assessed if combining FibroScan® measurement of liver stiffness transient elastography with ultrasound would improve the prediction of the development of a nodular liver on ultrasound and development of portal hypertension over time in children and young adults with CF. Study visits occurred at baseline and for at least two annual follow-up visits. This study is an ancillary study to the CFLD PUSH study. If requesting access to the ELASTIC-CF data, it is recommended to also request access to the CFLD PUSH data to obtain comprehensive information collected on study participants.