Cystic fibrosis associated liver disease in children

Table 4 Diagnostic criteria of cystic fibrosis liver disease

Debray et al[25]	CF foundation classification[24]
Hepatomegaly and/or splenomegaly- increased liver span at midclavicular line and spleen size in longitudinal coronal plane for age and sex, confirmed by ultrasonography	CF related liver disease with cirrhosis/portal hypertension (based on clinical exam/imaging, histology, laparoscopy)
Abnormalities of liver function tests-elevated AST and ALT and GGT levels above the upper limit of normal with at least at 3 consecutive determinations over 12 months after excluding other causes of liver diseases	Liver involvement without cirrhosis/portal hypertension consisting of at least one of the following: (1) Persistent AST, ALT, GGT > 2 times upper limit of normal; (2) Intermittent elevations of the above laboratory values; (3) Steatosis (histologic determination); (4) Fibrosis (histologic determination); (5) Cholangiopathy (based on ultrasound, MRI, CT, ERCP); and (6) Ultrasound abnormalities not consistent with cirrhosis
Ultrasonographic evidence of coarseness, nodularity, increased echogenicity, or portal hypertension	Preclinical: No evidence of liver disease on clinical examination, imaging or laboratory values
Liver biopsy showing cirrhosis

AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; GGT: Gamma glutamyl transpeptidase; CF: Cystic fibrosis; MRI: Magnetic resonance imaging; CT: Computed tomography; ERCP: Endoscopic retrograde cholangiopancreatography.