Liver nodularity occurs throughout the cystic fibrosis liver disease (CFLD) spectrum, from regenerating nodules to cirrhosis, and can develop in the absence of liver enzyme abnormalities. The goals were to see if combining abdominal ultrasonography (US) with yearly laboratory testing enhances CFLD diagnosis and to set CF-specific limits for liver screening labs. CF patients older than 6 years who were exocrine pancreatic-insufficient underwent an ultrasound with Doppler and shear wave elastography. Based on US results, patients were classified as Normal, Echogenic, or Nodular. The results were compared to aspartate aminotransferase (AST), alanine aminotransferase (ALT), platelets, the AST to platelet ratio index (APRI), fibrosis 4 (FIB-4), and the GGT to platelet ratio (GPR). The Youden Index was used to determine the receiver operator curve, sensitivity, specificity, positive predictive value, negative predictive value, and appropriate cut-off.
The addition of US detected more nodular livers in 82 cases than labs alone. The median AST (44), ALT (48), GGT (46), APRI (0.619), FIB-4 (0.286), and GPR were considerably higher in the Nodular group (1.431). AST>33, ALT>45, GGT>21, Platelets 230, APRI>0.367, FIB-4>0.222, GPR>0.682 were the optimal cut-offs for detecting hepatic nodularity in CF. We developed an algorithm to direct the usage of US in CFLD screening using GGT, APRI, and GPR.
Using adjusted serum lab thresholds, the inclusion of liver fibrosis markers, and/or the abdominal US, it is possible to identify liver nodularity in CF patients. A combination of GGT, GPR, and APRI can assist in determining which CF children should be evaluated in the United States. These technologies might aid in the early detection of fibrosis and/or cirrhosis in CF patients.
Reference:journals.lww.com/jpgn/Fulltext/2019/10000/New_Algorithm_for_the_Integration_of_Ultrasound.4.aspx
