UCSF

Background

New 2023 CF liver disease (CFLD) guidelines advocate for additional screening in people with cystic fibrosis (PwCF), including biennial abdominal ultrasound. As a first step towards effective and equitable guidelines implementation, we examined our current practice of CFLD screening and hepatobiliary involvement (HBI) evaluation. We identified characteristics of PwCF at-risk for incomplete screening and factors affecting evaluation.

Methods

We retrospectively reviewed medical records of PwCF aged 0-21 years, with native liver and ≥ 2 outpatient CF clinic visits 2017-2023. Logistic regression was used to identify characteristics associated with incomplete screening and with HBI.

Results

Amongst 112 PwCF at our center: 37% (n = 42) self-reported as mixed race, 27% (n = 30) as Hispanic; 53% (n = 59) had public insurance. Incomplete lab screening was identified in 19% of our cohort. GGT was the most frequently missed component (14%, n = 16). Hispanics and publicly insured people were more likely to have incomplete screening. Of the 112, 45 met criteria for HBI. Demographics did not predict HBI. Five with CF and HBI had the full hepatitis workup recommended by the new guidelines. Those with HBI documented (42%, n = 19) were more likely to receive additional workup. PwCF who were seen by a gastroenterologist were more likely to have additional diagnostic work-up for HBI.

Conclusion

One in five PwCF at our center were incompletely screened for CFLD, with Hispanics and publicly insured at higher risk. Accurate diagnosis and adequate documentation are the first steps to identifying HBI in PwCF. A dedicated CF gastroenterologist is key to completing CFLD screening and liver diagnosis.