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Liver Disease in Children with Cystic Fibrosis: US-Biochemical Comparison in 195 Patients¹

¹ From the Departments of Medical Imaging (H.P., G.P., A.G., D.F., J.B.) and Gastroenterology (C.L., L.S., C.C.R., A.R.W.), Sainte-Justine Hospital, 3175 Côte Ste-Catherine, Montreal, Quebec, H3T 1C5, Canada. Received December 24, 1997; revision requested March 24, 1998; revision received August 3; accepted October 13. Supported in part by grants from the Justine-Lacoste-Beaubien Foundation. Address reprint requests to H.P.

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; mean age, 8.5 years) attending a CF clinic underwent abdominal US and a standard set of liver function tests. Aspartate aminotransferase, alanine aminotransferase, and -glutamyltransferase levels were analyzed. US signs were interpreted as follows: hypoechogenicity with prominent portal tracks as edema, hyperechogenicity as steatosis, and increased attenuation and nodules within or at the edge of the liver as cirrhosis. Signs of portal hypertension also were sought. US signs were compared with liver function test results.

RESULTS: Liver sonograms were abnormal in 38 children (19%); of these, 24 (63%) had abnormal test results. The 157 children with normal liver architecture had a much lower prevalence of biochemical abnormality (33 patients [21%]; P .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 results of three liver function tests in children with CF was significant. The most specific US abnormalities related to abnormal function are signs suggestive of portal hypertension and cirrhosis.

Index terms: Fibrosis, cystic, 761.1496 • Hypertension, portal, 95.711 • Liver, cirrhosis, 761.794 • Liver, US, 761.12983 • Ultrasound (US), in infants and children, 761.12983

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