Gallbladder anomalies and cystic fibrosis

Area of Science:

• Pediatric Gastroenterology
• Medical Imaging
• Genetic Disorders

Background:

• Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the biliary system.
• Gallbladder abnormalities have been anecdotally reported in children with CF, but systematic investigation is limited.

Purpose of the Study:

• To investigate gallbladder size and morphology in children with cystic fibrosis (CF) using ultrasonography.
• To compare gallbladder findings in CF patients with age-matched healthy controls.
• To explore correlations between gallbladder abnormalities and clinical symptoms or treatment in CF.

Main Methods:

• Ultrasonography was performed on 46 children with CF and 45 controls.
• Gallbladder dimensions were assessed, categorizing them as micro-gallbladder or macro-gallbladder.
• Correlation with cholecystography, intravenous cholangiography, abdominal pain, steatorrhoea, and enzyme replacement dose was examined.

Main Results:

• Statistically significant differences in gallbladder size were observed in children with CF over 3 years of age compared to controls.
• Micro-gallbladders and macro-gallbladders were more prevalent in the pediatric CF cohort.
• Good correlation was found between ultrasonography, cholecystography, and intravenous cholangiography findings.
• No relationship was identified between abnormal gallbladders and abdominal pain, steatorrhoea, or enzyme replacement therapy dose.

Conclusions:

• Children with cystic fibrosis exhibit a higher incidence of abnormal gallbladder sizes (micro- and macro-gallbladders).
• Ultrasonography is a reliable method for assessing gallbladder abnormalities in CF, correlating well with other imaging techniques.
• Gallbladder size variations in CF children are not associated with common gastrointestinal symptoms or treatment intensity.