Description

A patient with cystic fibrosis may develop a variety of cystic fibrosis-associated liver diseases (CFALD). Some of these may be progressive, leading to portal hypertension and hepatic failure. A patient with advanced CFALD is at risk for hepatic encephalopathy.


 

Conditions that may precipitate hepatic encephalopathy in a patient with advanced CFALD:

(1) end-stage liver disease with liver failure

(2) infection

(2a) septicemia

(2b) pneumonia

(3) gastrointestinal disease

(3a) gastrointestinal bleeding, especially associated with portal hypertension

(3b) diarrhea, especially if it triggers an electrolyte imbalance

(3c) vomiting, especially if it triggers an electrolyte imbalance

(3d) distal intestinal obstruction syndrome (DIOS)

(4) fluid and electrolyte disorders:

(4a) acidosis

(4b) electrolye imbalances

(4c) forced diuresis

 

The hepatic encephalopathy may catch the patient, family and physician by surprise if the liver disease was not recognized or its severity was not appreciated.

 


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