Abstract
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 0-21yrs, with native liver and ≥2
outpatient CF clinic visits 2017-23. Logistic regression was used to
identify characteristics associated with incomplete screening and with
HBI. Results Amongst 112 PwCF at our center: 37% self-reported as mixed
race, 27% as Hispanic; 53% had public insurance. Incomplete lab
screening was identified in 19% of our cohort. GGT was the most
frequently missed component (17%). 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%) 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. Conclusions: 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.