Gastro hep advances · 2025
Effect of Cystic Fibrosis Transmembrane Conductance Regulator Modulators on Liver Enzymes Among Patients With Cystic Fibrosis: A Systematic Review and Meta-Analysis.
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Vongsavath Tahne, Tun Kyaw Min, Mukhopadhyay Dwaipayan, Lo Chun-Han, Singh Ashok, Amin Rajan, Twohig Patrick, Saab Sammy, Manne Vignan
Аннотация
BACKGROUNDS AND AIMS: This paper seeks to use existing literature to investigate the use of Cystic Fibrosis Transmembrane Conductance (CFTR) modulators and their safety and effects on the liver enzymes of cystic fibrosis (CF) patients with and without related liver disease (CFLD). This review examines the effect of CFTR modulators on liver enzymes as well as their safety profile.
METHODS: A comprehensive literature review across Pubmed/Medline, Embase, CINAHL, Cochrane, and Web of Science, was performed. Six articles pertaining to CFTR modulator use and liver enzyme information were included. Statistical evaluation was completed using random-effect models with a 95% confidence interval (CI), and group mean differences were analyzed with R software. Forest plots were generated for the observed effect, CI, and the weight of each study. The primary outcome was changes to serum levels of alanine transaminase (ALT), aspartate transaminase, total bilirubin, and gamma-glutamyl transferase. Secondary outcomes for this study were changes in serum biomarkers and type of CFTR modulator used. Other outcomes explored include the safety of CFTR modulator use.
RESULTS: Patients treated with lumacaftor/ivacaftor had a significant decrease in total bilirubin (-0.70 (-1.26, -0.14); P = .033) and gamma-glutamyl transferase (-0.98 (-1.45, -0.51); P = .012). While ALT had a nonsignificant trend (-0.56 (-1.54, 0.41); P = .20), ALT was noted to decrease significantly in CF patients (-0.90 (-1.71, -0.08); P = .037). When pooled there was significant change in ALT in those with and without CFLD (-0.98 (-1.45, -0.51); P = .012). Reported adverse events were low, with a prevalence of 38% (CI: 8.59%-80%).
CONCLUSION: CFTR modulators may influence levels of liver enzymes in CF patients without an increased risk of adverse events.
METHODS: A comprehensive literature review across Pubmed/Medline, Embase, CINAHL, Cochrane, and Web of Science, was performed. Six articles pertaining to CFTR modulator use and liver enzyme information were included. Statistical evaluation was completed using random-effect models with a 95% confidence interval (CI), and group mean differences were analyzed with R software. Forest plots were generated for the observed effect, CI, and the weight of each study. The primary outcome was changes to serum levels of alanine transaminase (ALT), aspartate transaminase, total bilirubin, and gamma-glutamyl transferase. Secondary outcomes for this study were changes in serum biomarkers and type of CFTR modulator used. Other outcomes explored include the safety of CFTR modulator use.
RESULTS: Patients treated with lumacaftor/ivacaftor had a significant decrease in total bilirubin (-0.70 (-1.26, -0.14); P = .033) and gamma-glutamyl transferase (-0.98 (-1.45, -0.51); P = .012). While ALT had a nonsignificant trend (-0.56 (-1.54, 0.41); P = .20), ALT was noted to decrease significantly in CF patients (-0.90 (-1.71, -0.08); P = .037). When pooled there was significant change in ALT in those with and without CFLD (-0.98 (-1.45, -0.51); P = .012). Reported adverse events were low, with a prevalence of 38% (CI: 8.59%-80%).
CONCLUSION: CFTR modulators may influence levels of liver enzymes in CF patients without an increased risk of adverse events.