Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral centre cohort

Early detection of hepatobiliary involvement in cystic fibrosis: Biomarkers, radiologic methods, and genetic influences

“…A report by the National Institutes of Health (NIH) proposed a modified criteria to evaluate CFHBI among adults that revealed 47% of their population had some degree of hepatic involvement and that CFHBI was more common among adults than previously reported. 18 Importantly, though, aCFLD remains a rare finding among adults, with single-center studies concluding that adult CFHBI is largely a benign condition [19][20][21][22] and well-illustrated by the French CF Modifier Gene Study 5 (see Figure 1). The primary challenge facing the CF clinician is the early identification of those patients that will progress to aCFLD compared to those with more benign forms of CFHBI.…”