213 Diagnosis of liver disease in children with cystic fibrosis: Are we getting it right?

Abstract

Objectives Cystic fibrosis associated liver disease (CFLD) is a common complication of cystic fibrosis (CF). Objective of this study was to identify factors associated with the development of liver disease in children with CF. Methods In a retrospective study of 178 children attending a single centre, cases were identified if they had an abnormal liver ultrasound on 2 separate occasions, then age and sex matched with 2 controls with normal ultrasound. Logistic regression analysis was performed to analyse the effect of a number of clinical parameters on liver disease as the outcome. Liver enzyme levels and medications at the time of diagnosis of CFLD were assessed. A request was submitted to UK CF Registry to retrieve data for 3809 patients less than 16 years of age on the UK CF registry for 2012. Results Ten cases were identified and matched with 20 controls. Surprisingly 9/20 (45%) controls with no study defined CFLD were on ursodeoxycholic acid (UDCA). UK CF Registry data showed that 12% of children without Registry defined CFLD were on UDCA. At the time of study-defined diagnosis of liver disease, Gamma glutamyl transferase (GGT) was significantly elevated in the cases compared to controls (mean 21.3 vs 11.7iu/L; p=0.03). Logistic regression analysis showed highest odds ratio (61.6) for homozygous DF508, although this was not statistically significant. Conclusions Many children with CF may be started on UDCA unnecessarily. Decisions about starting UDCA should be carefully considered, as overtreatment unnecessarily adds to the burden of care. This study suggests that GGT may have potential as an additional indicator assisting in diagnosis of CFLD.