Due to their relatively high abundance in CFLD patients and their pathophysiologic relation to the metabolism of extracellular matrix and/or angiogenesis as key events for the development of hepatic matrix deposition, we chose to further validate and quantify the serum levels of TIMP-4, Endoglin, Hepatocyte growth factor (HGF), and Pentraxin-3 (PTX3) in ELISA measurements in the whole CF cohort of 45 patients. Here, PTX3 is linked to cystic fibrosis-related liver disease.