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Background <p>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.</p> Methods <p>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.</p> Results <p>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.</p> Conclusions <p>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.</p>

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Evaluation of hepatobiliary involvement in children with cystic fibrosis: a retrospective single-center study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年8月5日

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  • Ahmet Uzger,
  • Ahmet Rauf Goktepe,
  • Pelin Asfuroğlu,
  • Yasin Sahin

摘要

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.