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A Review of Biliary Atresia
EP25389
Poster Title: A Review of Biliary Atresia
Submitted on 08 Feb 2017
Author(s): Veronica C. Varela, Stephanie C. Radu, Russell S. Kirby
Affiliations: University of South Florida
This poster was presented at USF Research Day 2017
Poster Views: 1,572
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Poster Information
Abstract: Biliary Atresia is a rare destructive, inflammatory condition in which progressive fibrosis of the biliary tree in infant’s leads to bile duct obstruction and subsequent liver cirrhosis. If left untreated progressive liver cirrhosis leads to death by the age of two. A comprehensive literature review was conducted in which,PubMed was used. References were reviewed from chosen articles to further identify additional sources. The review was limited to publications in English and did not include case reports. Biliary Atresia is one of the top reasons for liver transplantation in the pediatric population. As of January 2016, it was estimated that the incidence of Biliary Atresia in western countries, such as the United States, was 0.5-0.8 per 10,000 live births. There are several screening tools that have been discussed for the early identification of Biliary Atresia although many of the tests discussed have not been widely implemented. Taiwan, Canada, France and the UK have all implemented a stool color card as a screening tool for BA and have seen a significant reduction in the time of diagnosis and treatment for Biliary Atresia. The primary form of treatment for Biliary Atresia is a Kasai Portoenterostomy which has the strongest effectiveness if performed before the patient is 60 days of age.
Although Biliary Atresia is a rare birth defect in the U.S it remains one of the top causes for liver transplantation in infants. Effective screening tools such as the stool color card have been found to significantly reduce time to diagnosis as well as time to Kasai treatment. A reduction in diagnosis and treatment is especially important in order to reduce the likelihood of liver transplantation as well as to reduce complications from Kasai or liver transplantation. The etiology of Biliary Atresia is not yet fully understood; therefore a primary form of prevention is not possible at this time. Ensuring earlier diagnosis of Biliary Atresia can improve survival significantly.
Summary: A literature review was conducted to gather information on Biliary Atresia (BA).The incidence of BA in the U.S, is 0.5-0.8 per 10,000 live births. The U.S currently doesn’t have a screening tool implemented. Many countries have implemented a stool color screening tool which has proven effective in early diagnosis of BA. Although BA is rare in the U.S it is a top causes for liver transplantation in infants. Opportunities for using a screening tool in the U.S for BA should be further explored.References: 1. Lee S, Park H, Moon SB, et al. Long-term results of biliary atresia in the era of liver transplantation. Pediatr Surg Int.
2013;29(12):1297-1301.
2. Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009;374(9702):1704-1713.
3. Nizery L, Chardot C, Sissaoui S, et al. Biliary atresia: Clinical advances and perspectives. Clin Res Hepatol Gastroenterol.
2016;40(3):281-287.
4. Chiu CY, Chen PH, Chan CF, Chang MH, Wu TC, Taiwan Infant Stool Color Card Study G. Biliary atresia in preterm
infants in Taiwan: a nationwide survey. The Journal of pediatrics. 2013;163(1):100-103 e101.
5. Wada H, Muraji T, Yokoi A, et al. Insignificant seasonal and geographical variation in incidence of biliary atresia in
Japan: a regional survey of over 20 years. J Pediatr Surg. 2007;42(12):2090.
6. Girard M, Jannot AS, Besnard M, et al. Polynesian ecology determines seasonality of biliary atresia Hepatology.
2011;54:1893.
7. The NS, Honein MA, Caton AR, Moo
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