Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease

Background: The objective of our study was to determine if hepatic ultrasound findings in paediatric patients with cystic fibrosis and suspected liver disease are related to histopathological results derived from liver biopsies. Methods: A retrospective analysis of ultrasound and liver biopsy findings using published criteria was performed in 30 children with cystic fibrosis suspected as having liver disease on clinical, biochemical and ultrasonographic criteria. The results were correlated and assessed for intra- and interobserver agreement. Results: A significant association was found for the prediction of fibrosis or cirrhosis on the basis of ultrasound (p=0.03). There was no significant association between normal or indeterminate ultrasound and histology results. A high intra- and interobserver variability was found in sonographic assessment of the hepatic echostructure. Conclusions: The diagnosis of early liver disease in cystic fibrosis cannot reliably be made on the basis of ultrasound alone. A normal ultrasound does not preclude significant liver fibrosis in cystic fibrosis. An abnormal US that suggests cirrhosis predicts the presence of moderate to severe liver disease. Crown Copyright (C) 2007 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. All rights reserved.