Review of historical cohort: ursodeoxycholic acid in extrahepatic biliary atresia Magd A. Kotb ⁎ Department of Pediatrics, Cairo University, PO Box, 85 El Mokatam, Cairo 11571, Egypt Received 5 August 2007; revised 29 November 2007; accepted 30 November 2007 Key words: Ursodeoxycholic acid; Extrahepatic biliary atresia; Outcome Abstract Background: Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hepatocytes, and dissolve gallstones. Purpose: The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia. Methods: We used a statistical analysis of data of records of infants and children having extrahepatic biliary atresia who underwent Kasai portoenterostomy and attended Hepatology Clinic, New Children's Hospital, Cairo University, Egypt, from May 1985 until June 2005. Results: Of 141 infants with extrahepatic biliary atresia, 108 received ursodeoxycholic acid for mean duration ± SD of 252.6 ± 544.9 days in a dosage of 20 mg/kg per day. The outcome of infants who did not receive ursodeoxycholic acid and those who did was the following: 8 (24.2%) and 11 (10.18%) had a successful outcome (P = .043), 0 (0%) and 7 (6.4%) improved (P = .148), 25 (75.7%) and 84 (77.7%) had a failed outcome (P = .489), and none vs 5 died (4.6%) (P = .135), respectively. The predictors of successful outcomes were age less than 65 days at portoenterostomy (P = .008) and absence of ursodeoxycholic acid intake (P = .04) with a likelihood of a successful outcome that was 2.8, that associated with ursodeoxycholic acid intake. Conclusion: In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications. © 2008 Elsevier Inc. All rights reserved. Extrahepatic biliary atresia is a progressive, sclerosing, obstructive inflammatory process in neonates, causing atresia of all or part of the extrahepatic biliary system and rapidly extending to involve the major intrahepatic bile ducts [1]. Palliative surgery such as a Kasai portoenterostomy attempts to establish bile flow in extrahepatic biliary atresia. The success of this procedure is greatest when performed within the first 2 months of life, but it is curative only in a minority of cases [2]. More commonly, 68% to 80% of the children with biliary atresia have deterioration in their liver function and eventually need a liver transplant [3-6]. Ursodeoxycholic acid is a hydrophilic physiologic dihydroxy bile acid that was first identified in the bile of a Chinese black bear [7] and present in man in a concentration of approximately 3% of the bile acid pool. It is formed by 7b-epimerization of primary bile acid chenodeoxycholic acid in the gut by intestinal bacteria [8,9]. Ursodeoxycholic acid intake in various cholestatic and gall stone diseases was ⁎ Tel.: +20 002 010 1420 831; fax: +20 002 02 2403 7981. E-mail address: magdkotb@hotmail.com. www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.11.043 Journal of Pediatric Surgery (2008) 43, 1321–1327