Cystic fibrosis in the liver

Question: A teenage male with cystic fibrosis (CF) is sent to see you because of a cholestatic liver profile, and you are asked whether he needs to worry about CF in his liver.  What is the likelihood that he experiences liver disease from this genetic condition?

A. 5%

B. 30%

C. 75%

D. CF does not cause liver disease

Answer: These days, median survival of patients with CF is almost 50 years.  Liver disease is the third cause of death in American patients with CF.  Two of the roles of the CFTR (chloride channel) is to keep the canalicular bile contents alkalinized and at the right viscosity.  If viscosity rises, bacterial counts rise and an inflammatory/fibrotic reaction ensues.  So, we can eliminate D from our answer choices.

The prevalence of CF liver disease is actually between 27-41%, depending on the article you review.  It is usually an early complication of CF, developing before age 20.  This patient may be experiencing some liver disease based on his cholestatic labs.  The influence of liver disease on the overall clinical course of CF remains unclear.  It can progress to cirrhosis and portal hypertension, and liver + lung transplants have occurred. 

How should this patient be managed, from a liver perspective? Good question.  There is no great answer.  CF liver lesions are often focal, so liver biopsy may be misleading.  It is likely that if this patient grows into adulthood without developing cirrhosis, the liver component of his disease will be clinically benign.

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