Evaluation of hepatobiliary involvement in children with cystic fibrosis: a retrospective single-center study.
4 affiliations
- 1Department of Pediatric Gastroenterology, Gaziantep City Hospital, Gaziantep, Gaziantep, 27560, Turkey.
- 2Clinic of Pediatric Pulmonology, Gaziantep City Hospital, Gaziantep, Gaziantep, 27560, Turkey.
- 3Department of Pediatric Gastroenterology, Gaziantep City Hospital, Gaziantep, Gaziantep, 27560, Turkey. [email protected].
- 4Faculty of Medicine, Gaziantep Islam Science and Technology University, Gaziantep, Gaziantep, 27560, Turkey. [email protected].
Abstract
Background
It is difficult to know the actual prevalence of liver disease in patients with cystic fibrosis (CF) because of the lack of an accepted definition of cystic fibrosis-associated liver disease (CFALD), the fact that most patients are asymptomatic, and the lack of specific noninvasive diagnostic tests. This study aimed to investigate the frequency and characteristics of liver involvement in patients diagnosed with CF.Methods
This retrospective study was conducted from January 2024 to January 2025. Pediatric patients aged 1 - 18 years diagnosed with CF were included. All patients were evaluated for clinical and laboratory findings related to liver disease. Only patients with liver function test results and/or ultrasonography (US) findings were included in the study regardless of symptoms. Children with comorbid diseases other than CF, such as chronic liver disease, kidney disease, cerebral palsy, or type 1 diabetes mellitus, were excluded.Results
Sixty patients were included in the study. The mean age was 7.17 ± 5.69 (0.4-18.0) years. 31 (51.7%) of the patients were male. The mean ALT values of patients were 29.91 ± 23.52 IU/L. Ultrasound imaging was not performed in 13 patients. Among the 47 patients who underwent ultrasonography, 13 had abnormal findings and 34 had normal findings. Ten patients (16.6%) had abnormal liver function tests during follow-up. Five patients had hepatomegaly, six had a heterogeneous granular appearance, and two had hepatosteatosis. Three patients with a heterogeneous appearance on ultrasound had elevated serum transaminase levels. All patients with hepatomegaly, except one, had normal serum transaminase levels. Furthermore, one patient underwent a liver biopsy, and the biopsy findings were consistent with cirrhosis.Conclusions
Hepatobiliary involvement was detected in 18 (30.0%) patients. Elevated transaminase levels were not significantly associated with abnormal ultrasonographic findings. Our findings support regular liver disease screening in children with CF, including abdominal ultrasonography, to facilitate early recognition and management.
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BibTeX
@article{uzger2026evaluation,
title = {{Evaluation of hepatobiliary involvement in children with cystic fibrosis: a retrospective single-center study.}},
author = {Ahmet Uzger and Ahmet Rauf Goktepe and Pelin Asfuroğlu and Yasin Sahin},
journal = {BMC gastroenterology},
year = {2026},
month = aug,
doi = {10.1186/s12876-026-05186-6},
url = {https://europepmc.org/article/MED/42968009},
note = {CC BY},
}
RIS
TY - JOUR
TI - Evaluation of hepatobiliary involvement in children with cystic fibrosis: a retrospective single-center study.
AU - Ahmet Uzger
AU - Ahmet Rauf Goktepe
AU - Pelin Asfuroğlu
AU - Yasin Sahin
PY - 2026
DA - 2026/08/05
JO - BMC gastroenterology
DO - 10.1186/s12876-026-05186-6
UR - https://europepmc.org/article/MED/42968009
AB - Background It is difficult to know the actual prevalence of liver disease in patients with cystic fibrosis (CF) because of the lack of an accepted definition of cystic fibrosis-associated liver disease (CFALD), the fact that most patients are asymptomatic, and the lack of specific noninvasive diagnostic tests. This study aimed to investigate the frequency and characteristics of liver involvement in patients diagnosed with CF.Methods This retrospective study was conducted from January 2024 to January 2025. Pediatric patients aged 1 - 18 years diagnosed with CF were included. All patients were evaluated for clinical and laboratory findings related to liver disease. Only patients with liver function test results and/or ultrasonography (US) findings were included in the study regardless of symptoms. Children with comorbid diseases other than CF, such as chronic liver disease, kidney disease, cerebral palsy, or type 1 diabetes mellitus, were excluded.Results Sixty patients were included in the study. The mean age was 7.17 ± 5.69 (0.4-18.0) years. 31 (51.7%) of the patients were male. The mean ALT values of patients were 29.91 ± 23.52 IU/L. Ultrasound imaging was not performed in 13 patients. Among the 47 patients who underwent ultrasonography, 13 had abnormal findings and 34 had normal findings. Ten patients (16.6%) had abnormal liver function tests during follow-up. Five patients had hepatomegaly, six had a heterogeneous granular appearance, and two had hepatosteatosis. Three patients with a heterogeneous appearance on ultrasound had elevated serum transaminase levels. All patients with hepatomegaly, except one, had normal serum transaminase levels. Furthermore, one patient underwent a liver biopsy, and the biopsy findings were consistent with cirrhosis.Conclusions Hepatobiliary involvement was detected in 18 (30.0%) patients. Elevated transaminase levels were not significantly associated with abnormal ultrasonographic findings. Our findings support regular liver disease screening in children with CF, including abdominal ultrasonography, to facilitate early recognition and management.
SN - 1471-230X
ER -