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Gallbladder disease in cystic fibrosis
M C Jebbink1, H G Heijerman, A A Masclee
1Department of Gastroenterology and Hepatology, University Hospital Leiden, Netherlands.
Insights
Cystic fibrosis (CF) patients frequently develop gallbladder issues like gallstones and nonfunctioning gallbladders. Understanding the causes and improving surgical options are crucial for managing these gastrointestinal complications.
Area of Science:
- Gastroenterology
- Hepatology
- Pediatric Medicine
Background:
- Gallbladder abnormalities are common in cystic fibrosis (CF) patients.
- Prevalence includes nonfunctioning gallbladders (30%), micro-gallbladders (8-30%), and gallstones (4-30%).
- Pathophysiology remains unclear due to conflicting data on bile acid metabolism, nucleation time, and biliary stasis.
Purpose of the Study:
- To review the pathophysiology and diagnosis of gallbladder disease in CF.
- To discuss current treatment options and associated risks.
- To explore potential alternative surgical approaches.
Main Methods:
- Literature review of gallbladder abnormalities in CF patients.
- Analysis of pathophysiology, diagnostic challenges, and treatment outcomes.
- Discussion of surgical interventions and their implications.
Main Results:
- High prevalence of gallbladder dysfunction and gallstones in CF.
- Diagnostic delays are common due to overlapping GI symptoms.
- Cholecystectomy carries significant risks (10% morbidity, 5% mortality) in CF patients.
Conclusions:
- Gallbladder disease is a significant gastrointestinal complication in CF.
- Careful pre- and perioperative management is essential for cholecystectomy.
- Laparoscopic cholecystectomy warrants further investigation as a potentially safer alternative.
Abstract:
Among the various gastrointestinal manifestations observed in patients with cystic fibrosis (CF), gallbladder abnormalities occur frequently. These include a high prevalence of nonfunctioning gallbladders (30%), micro-gallbladders (8-30%), and gallstones (4-30%). The underlying pathophysiology for this increased prevalence in patients with CF is not completely understood, due to contradictory findings. These findings concern: (1) abnormalities in bile acid metabolism resulting in bile that is supersaturated with cholesterol, (2) an impaired nucleation time, and (3) biliary stasis, due to bile duct abnormalities and/or impaired gallbladder motility. The diagnosis of gallbladder disease in CF may be obscured by other common gastrointestinal complications, resulting in a long delay between onset of symptoms and the diagnosis. Cholecystectomy in CF is the treatment of choice, provided they are carefully managed in the pre- and perioperative period. The operative morbidity and mortality, even with intensive management of pulmonary disease, amounted to 10% and 5%, respectively. Therefore, alternative options, like laparoscopic cholecystectomy are of interest and require further investigation, especially for CF patients with severe pulmonary disease.