Lives unaffected by cystic fibrosis

Gallbladder

It has long been recognized that patients with cystic fibrosis (CF) can develop gallbladder abnormalities. This may include small or absent gallbladder, gallbladder dysfunction, symptomatic cholelithiasis, and malignancy.

CF has been associated with higher rates of abnormal gallbladder function and higher incidence of gallstones. The gallbladder represents a frontier of research in the world of cystic fibrosis because new screening techniques and diagnostics are becoming available. 

Early diagnosis of CF is crucial because early interventions and treatments have proven the most effective. Gallbladder evaluation in utero may provide a powerful tool in the early identification and treatment of CF.

Gallbladder evaluation is best conducted via ultrasound, which has the added benefit of being less invasive than certain other diagnostic measures.

Black pigmented gallstones are more common in individuals who are managing CF, this contrasts with the general population in which cholesterol gallstones are more prevalent.

Assessment of gallbladder problems in individuals who are managing CF is complicated by the large number of additional and overlapping ailments which can result from CF. This often poses the danger of disguised complications that can go unnoticed until they pose a serious risk.

Biliary disorders, including gallbladder and bile duct disease, are common complications of cystic fibrosis. The gallbladder stores bile produced by the liver. Bile aids in the digestion of fat from the diet. After meals, the gallbladder squeezes stored bile into the small intestine through a series of tubes called ducts. As a result, symptoms of gallbladder disease tends to be sharp, localized abdominal pain, especially after meals. 

CF has been associated with higher rates of abnormal gallbladder function and higher incidence of gallstones (cholelithiasis) and inflammation of the gallbladder (cholecystitis). In cystic fibrosis the cells in the gallbladder have a reduced ability to regulate fluid and chloride transport, which leads to problems with the secretion and function of bile. This means the bile can become sticky, causing irritation and inflammation in the ducts and potentially obstructing bile ducts by the formation of gallstones.

Microgallbladder, which has been reported in 25-45% of individuals with CF in some studies, describes a very small gallbladder as diagnosed by ultrasound. On its own it is asymptomatic, but is significant because it is often identified in association with cholecystitis and cholelithiasis.

Assessment of gallbladder problems in individuals who are managing CF can be complicated by the fact that many of the symptoms are similar to other ailments commonly faced with CF. Currently, the best method to evaluate the gallbladder is via ultrasound, which has the added benefit of being less invasive than certain other diagnostic measures. 

The gallbladder represents a frontier of research in the world of cystic fibrosis because new screening techniques and diagnostics are becoming available. It is unknown whether current and next-generation CFTR correctors can influence the formation of gallstones or other gallbladder conditions in patients with CF.

CF and Gallbladder complications:

https://www.cysticfibrosisjournal.com/article/S1569-1993(17)30821-4/fulltext

https://pubmed.ncbi.nlm.nih.gov/1470281/

https://www.hindawi.com/journals/crira/2019/6737428/

https://www.cff.org/CF-Community-Blog/Posts/2019/Things-I-Learned-After-Gallbladder-Surgery/

https://www.uptodate.com/contents/cystic-fibrosis-hepatobiliary-disease

https://link.springer.com/article/10.1007/s11894-005-0053-6

https://aasldpubs.onlinelibrary.wiley.com/doi/pdf/10.1002/hep.1840140505