A patient with cystic fibrosis (CF) may develop hepatobiliary disease, which may include disorders of the distal bile duct.


The patient may present with:

(1) right upper quadrant pain

(2) intermittent or persistent jaundice and/or elevated total serum bilirubin

(3) elevated serum alkaline phosphatase with variable elevation of transaminases

(4) elevated serum bile acids


Conditions that may affect the distal bile duct in a patient with CF:

(1) cholelithiasis

(2) duct obstruction due to fibrosis in chronic pancreatitis

(3) common bile duct stricture

(4) sclerosing cholangitis (rare)

(5) cholangiocarcinoma (rare)


Workup may include:

(1) ultrasonography

(2) endoscopic retrograde cholangiopancreatography (ERCP)

(3) bile duct cytology


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