Diagnosis and predicted outcomes of patients with cystic fibrosis related liver disease considered for lung transplantation

Espeed Khoshbin*, Stephen Clark, Gerard Meachery, Andrew Fisher, Anthony De Soyza, James Lordan, Arun Nair, John Dark, Mark Hudson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


Introduction: 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. 

Method: Lung recipients (n = 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. 

Result: 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. 

Conclusion: 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.

Original languageEnglish
Article numbere14713
Number of pages7
JournalClinical Transplantation
Issue number8
Early online date19 May 2022
Publication statusPublished - 1 Aug 2022

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