Advances in cystic fibrosis (CF) therapy have increased survival and, as a result, the incidence of individuals with liver disease, making chronic liver disease one of the most serious consequences of CF. The study presents the incidence of liver fibrosis, portal hypertension, and liver decompensation using an expanded panel of biochemical markers and ultrasonography for cystic fibrosis-related liver disease (CFLD). The Copenhagen CF Center conducted a cross-sectional investigation of CFLD in all juvenile CF patients. Physical examination, biochemical analysis, Vibration-Controlled Transient Elastography, conventional ultrasonography, and Real-Time Shear Wave elastography were all used to screen for liver disease (SWE). Within 6 months, patients were assessed using the Williams ultrasonography grading scale. 84 patients were involved in the study. In this investigation, eight individuals were classified as having evident CFLD because they had two aberrant sonographic results and two abnormal biochemical data. Patients with manifest CFLD were substantially older and had a higher mean APRI value, but no differences in gender, z-height, z-weight, z-BMI, FEV1 percent, or mean bilirubin or albumin values were identified.
According to both biochemical and sonographic testing, 8 individuals (9.5 percent) in this juvenile CF group were diagnosed with CFLD. The results of FibroScan and SWE were consistent. As CFLD diagnostic indicators, we recommend WUSS, FibroScan, or SWE in combination with GGT.
