Cycstic Fibrosis Guidelines (Updated 2023)

- Annual Labs starting at the time of CF diagnosis: Total Bili, AST, ALT, Alk Phos, GGT, Plt

- Annual physical exam to detect hepatosplenomegaly

- Abd US to assess liver and spleen every 2yrs

- CF patients with Hepatobiliary involvement (abnormal physical exam, persistently elevated liver enzymes >3-6mo, abnormal US findings) should undergo baseline liver elastography

- CF patients with hepatobiliary involvement should undergo a standardized evaluation for other causes of liver disease (r/o Chronic Hepatitis, cholestasis, autoimmune liver disease, alcoholic and NAFLD, A1AT, Wilson, Celiac, DILI)

- CF patients with evidence of cholestasis, abd pain, and inconclusive abd US should undergo MRCP to detect hepatolithiasis, choledocholithiasis, biliary strictures, and/or biliary obstruction