Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients

PURPOSE: To determine if abnormal liver architecture at ultrasonography (US ) is related to abnormal function in children with cystic fibrosis (CF). MATERIALS AND METHODS: For 1 year, all 195 children (112 boys, 83 girls; me an age, 8.5 years) attending a CF clinic underwent abdominal US and a stand ard set of liver function tests. Aspartate aminotransferase, alanine aminot ransferase, and gamma-glutamyltransferase levels were analyzed. US signs we re interpreted as follows: hypoechogenicity with prominent portal tracks as edema, hyperechogenicity as steatosis, and increased attenuation and nodul es within or at the edge of the liver as cirrhosis. Signs of portal hyperte nsion also were sought. US signs were compared with liver function test res ults. RESULTS: Liver sonograms were abnormal in 38 children (19%); of these, 24 ( 63%) had abnormal test results. The 157 children with normal liver architec ture had a much lower prevalence of biochemical abnormality (33 patients [2 1%]; P less than or equal to .001). All eight children with signs of portal hypertension had abnormal test results. Fourteen (82%) of 17 children with signs of cirrhosis had abnormal liver function. Eight (57%) of 14 patients with signs of steatosis had abnormal function. Diffuse hypoechogenicity of the liver with prominent portal tracks in 16 patients was associated with abnormal function in only five patients. CONCLUSION: The relation between abnormal liver architecture at US and resu lts of three liver function tests in children with CF was significant. The most specific US abnormalities related to abnormal function are signs sugge stive of portal hypertension and cirrhosis.