Description:
<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction</jats:title><jats:p>There is no gold standard criterion for the diagnosis of cystic fibrosis‐related liver disease (CFRLD) and there is uncertainty over its impact on the outcome of lung transplantation.</jats:p></jats:sec><jats:sec><jats:title>Method</jats:title><jats:p>Lung recipients (<jats:italic>n</jats:italic> = 238) were divided into two groups—CFRLD and non‐CFRLD based on a modified aspartate aminotransferase‐to‐platelet ratio index (APRI) score (mAPRI) to diagnose CFRLD and predict severity of liver disease. Groups were compared to assess validity of the diagnosis and survival outcomes.</jats:p></jats:sec><jats:sec><jats:title>Result</jats:title><jats:p>The new diagnostic criterion was effective at differentiating CFRLD from non‐CFRLD. There was no significant difference in the survival between two groups at short, medium, or long term demonstrated by the Kaplan‐Meier plot with survival of 85%, 73%, 47%, 18.6%, and 4.7% at 1, 2, 5, 10, and 15 years respectively. A mAPRI score of greater than .2 had a sensitivity of 43.0% but a specificity of 82.5 % for diagnosis of CFRLD and 46.5% sensitivity but 100% specificity in predicting an ultrasound/biopsy proven hepatic abnormality associated with CFRLD.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>A mAPRI sore is a highly specific non‐invasive tool for diagnosis of CFRLD. Recipients with CFRLD but grossly preserved hepatocellular function have a similar outcome to patients without CFRLD.</jats:p></jats:sec>