Published online November 24, 2008
PEDIATRICS (doi:10.1542/peds.2008-0986)
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ARTICLE

Effect of Ursodeoxycholic Acid on Liver Function in Children After Successful Surgery for Biliary Atresia

Stephanie Willot, MDa, Stephanie Uhlen, MDa, Laurent Michaud, MDa, Gilbert Briand, MD, PhDb, Michel Bonnevalle, MDc, Rony Sfeir, MDc and Frédéric Gottrand, MD, PhDa

a Departments of Pediatric Gastroenterology, Hepatology, and Nutrition
c Pediatric Surgery, Lille University Children's Hospital, Lille, France
b Biochemistry Laboratory, Site Eurasanté, Lille University Hospital Center, Lille, France

OBJECTIVES. Although ursodeoxycholic acid has been used to treat various cholestatic liver diseases in children, few data are available about its efficacy in biliary atresia. The aim of this study was to assess the effect of ursodeoxycholic acid treatment on liver function in children who underwent successful surgery for biliary atresia.

PATIENTS AND METHODS. We prospectively studied 16 children with biliary atresia who underwent successful portoenterostomies (postoperative conjugated bilirubin concentration: <34 µmol/L) and were treated with ursodeoxycholic acid for at least 18 months after surgery. Ursodeoxycholic acid treatment was then discontinued. Clinical and biological assessment was performed at the time of discontinuation of ursodeoxycholic acid treatment (T0), at follow-up (T1) and, if the clinical or biological status worsened, after resumption of ursodeoxycholic acid treatment (T2).

RESULTS. Ursodeoxycholic acid treatment was resumed in 13 cases. In 1 patient, jaundice recurred after ursodeoxycholic acid therapy was discontinued but abated after resumption of treatment. In 13 children, liver function worsened significantly when ursodeoxycholic acid was discontinued. T1 versus T0 concentrations expressed as multiples of the upper limit of the normal range (in parentheses) were as follows: alanine aminotransferase, 3.0 xN (0.8–7.0) vs 1.5 xN (0.5–5.4); {gamma} glutamyl transpeptidase, 8.0 xN (1.8–30.2) vs 4.2 xN (0.5–27.4); and aspartate aminotransferase, 1.7 xN (0.7–6.0) vs 1.3 xN (0.6–3.4). When ursodeoxycholic acid treatment was resumed, liver function had improved in all patients by T2. Concentrations of endogenous bile acids tended to be elevated at T1 (not significant) and were significantly decreased at T2.

CONCLUSION. Our study demonstrates the beneficial effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia.

Key Words: biliary atresia • ursodeoxycholic acid • cholestasis • bile acids • portoenterostomy • liver enzymes • cirrhosis

Abbreviations: BA—biliary atresia • UDCA—ursodeoxycholic acid • ALT—alanine aminotransferase • AST—aspartate aminotransferase • GGT—{gamma} glutamyl transpeptidase

Accepted Aug 20, 2008.