Background: Cystic fibrosis (CF) is an autosomal recessive
disease affecting multiple organ systems, including the liver, leading
to cystic fibrosis-related liver disease (CFLD). It was noted that CFLD
in Egyptian children with CF is more common than in non-Egyptian people
with CF (pwCF). The present study aimed to determine the incidence of
CFLD and the potential risk factors for developing CFLD in Egyptian
children. The correlation between CFLD and the various genotypes
prevalent in Egyptian CF children and the comparison of CFLD in Egyptian
and non-Egyptian CF patients will be done. Methods: The current
cross-sectional study included 50 CF cases from Ain Sham University’s
Pediatric Pulmonology Clinic in Children‘s Hospital, Cairo, Egypt. The
sweat chloride test and genetic studies were done at the time of
diagnosis. All patients’ caregivers provided informed consent.
Additionally, all subjects underwent detailed history taking, laboratory
investigations, clinical assessment, and pelviabdominal ultrasound for
evaluation of hepatic involvement. Results: Male sex, severe
genetic mutation (class I and II), long duration, and early onset of the
disease were independent risk factors for CFLD development. In addition,
pancreatic insufficiency, as well as meconium ileus history, were
predictors of CFLD. Diabetes mellitus and severe lung disease were
proven to significantly elevate the risk of CFLD development.
Conclusion: CFLD is not uncommon in Egyptian CF patients as
one-third of the patients were found to have liver affection. CF
patients with multiple risk factors are at increased risk of developing
liver disease.