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)
where:
• 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.
