Colombo et al listed criteria for liver disease in cystic fibrosis (CF).


Patient selection: cystic fibrosis


Criteria for the diagnosis of liver disease - 1 or both of the following:

(1) 2 or 3 of the following on 2 or more occasions spanning 1 year:

(1a) hepatomegaly (liver span in midclavicular line > 2 cm below the costal margin, confirmed on imaging studies)

(1b) abnormal liver function tests (at least 2 > than upper limit of normal)

(1c) abnormalities other than hepatomegaly seen on imaging studies (nodularity, irregular margins, splenomegaly, heterogeneous echogenicity)

(2) abnormal liver histology (biliary cirrhosis, cholangitis, etc)



• Liver function tests include: GGT, ALT, AST.


The diagnosis of CF-associated liver disease requires exclusion of other explanations for the positive findings.


The presence of unexplained liver disease in a pediatric patient or young adult should cause a clinician to consider the diagnosis of cystic fibrosis.


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