Improving detection of cystic fibrosis related liver disease using liver fibrosis assessment tools

• DOI: 10.1016/j.heliyon.2023.e21861
• Publication Date: 2023-11-07T19:58:40Z
• Authors: Jennifer A. Scott; Andrew M. Jones; Elliot Jokl; Timothy Gordon-Wa...; Peter J. Barry; Neil A. Hanley; Karen Piper Hanley; Varinder S. Athwal
• Keywords: Liver disease; Transient elastography

Cystic Fibrosis related liver disease (CFLD) is the 3rd largest cause of death in (CF). As advances pulmonary therapies have increased life-expectancy, CFLD has become more prevalent. Current guidelines may underdiagnose fibrosis, particularly its early stages. Newer modalities for assessment fibrosis provide a accurate assessment. FibroScan validated assessing several aetiologies including alcohol and fatty liver, cohort an entirely different phenotype so cut off values are not transferrable. We appraised tools to improve diagnosis CFLD.A prospective (n = 114) patients from Manchester Adult Centre, UK were identified at annual Demographic data co-morbidity, CFTR genotyping, biochemistry imaging used alongside current group into CF without evidence disease. All underwent stiffness measurement (LSM) serum-based biomarker panels. A new diagnostic criterion was created second, independent cohort.12 114 patient classified as according European Society best practice guidelines. No specific risk factors development identified. Liver enzymes elevated with CFLD. Serum panels did criteria. LSM accurately predicted proposed separate cohort, predicating 10 32 (31 %).We present assessed presence using blood biomarkers based platforms. propose new, simplified criteria, capable predicting CF.Clinical trials number: NCT04277819.