In a case control study, Pereira et al. demonstrated that elevated tissue inhibitor of matrix metalloproteinase (TIMP)-1, collagen (CL)-IV, and prolylhydroxylase (PH) may be indicators of hepatic fibrogenesis in CF, and increased TIMP-1, and PH may be early markers of CFLD [14]. This evidence concerns the gene TIMP1 and cystic fibrosis-related liver disease.