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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Specialty: Gastroenterology, Pulmonology
