Cystic fibrosis associated liver disease in children

Table 2 Causes of acute or chronic liver disease in cystic fibrosis patients showing hepatic abnormalities

Condition	Investigation
Acute/chronic viral hepatitis	Serology for HAV, HBV, HCV, EBV, CMV, adenovirus, HHV 6, parvovirus
α1 antitrypsin deficiency	Serum α1 antitrypsin level, including phenotype
Autoimmune hepatitis	Non-organ specific autoantibodies (SMA, anti-LKM1, LC1)
Celiac disease	Total IgA, IgA anti-tissue transglutaminase
Wilson disease	Ceruloplasmin, serum copper, 24 h urinary copper
Drug induced liver injury	Antibiotics (cyclines, macrolides, amoxicillin-based, and cephalosporins) & antifungals (azoles and polyenes)
Genetic hemochromatosis (adults)	Iron, Ferritin, Transferrin binding capacity
Other causes of steatosis	Malnutrition, diabetes, obesity

This table is modified from Debray et al[25]. HAV: Hepatitis A virus; HBV: Hepatitis B virus; HCV: Hepatitis C virus; CMV: Cytomegalovirus; EBV: Epstein-Barr virus; HHV6: Herpes hominis virus type 6; SMA: Smooth muscle antibody; LKM1: Liver kidney microsomal type 1; LC: Liver cytosol type 1; IgA: Immunoglobulin A.