The study investigated the influence of partial external biliary diversion (PEBD) surgery on clinical outcomes in individuals with increasing familial intrahepatic cholestasis (PFIC). Researchers conducted a systematic review of the literature (PubMed) and a meta-analysis to assess the relationships between liver biochemistry parameters (serum bile acids, bilirubin, and alanine aminotransferase [ALT]) and early response or long-term outcomes in PFIC patients who underwent PEBD. Prior to September 2018, 175 publications were found through searches; 16 of them satisfied the inclusion requirements. The potential of liver biochemistry markers to differentiate between patients who showed early and long-term response to PEBD was investigated using receiver operating characteristic (ROC) analysis. In terms of pruritus improvement, 104 of the 155 individuals included in the study responded, 14 responded somewhat, and 37 did not react. In ROC analyses of individual patient data, post-PEBD blood concentrations of bile acids, in particular, could differentiate responders from nonresponders for pruritus improvement; to a lesser extent, this was also true for bilirubin, but ALT could not. Reductions in blood bile acid concentration and bilirubin, but not ALT, from pre-PEBD levels substantially discriminated against the need for a liver transplant.
Changes in bile acids appear to be especially effective in distinguishing early and long-term post-PEBD outcomes and may represent possible indicators of responsiveness to enterohepatic circulation disruption in individuals with PFIC.