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Medication

Medication Summary

In patients with chronic cholestatic conditions and bile duct patency, ursodeoxycholic acid (ie, ursodiol, UDCA) has also been shown to enhance bile flow.^[23]For infants following portoenterostomy, UDCA may improve outcomes, and the drug was previously thought to be associated with minimal toxicity. However, one recent study evaluating UDCA in adults with primary sclerosing cholangitis found that long-term, high-dose treatment was associated with a higher rate of severe adverse events, including progression to cirrhosis. While the published experience with UDCA in biliary atresia has not shown similar deleterious outcomes, patients receiving long-term therapy with this agent should be carefully monitored.^[24]

In order to prevent cholangitis postoperatively, prophylaxis with trimethoprim-sulfamethoxazole has been used on a long-term basis. Unfortunately, conclusive data supporting the use of this agent, or the other drugs described above, in the management of biliary atresia are not available.

In years past, high-dose methylprednisolone was used in the immediate postoperative period. Results from the START (Steroids in Biliary Atresia Randomized Trial) study was not superior to surgery alone. The trial concluded that the proportion of infants with biliary atresia who underwent hepatoportoenterostomy, high-dose corticosteroids following surgery did not result in statistically significant treatment differences in bile drainage at 6 months, although a small clinical benefit could not be excluded. Also, corticosteroid therapy was associated with earlier onset of serious adverse events in children with biliary atresia.^[21]

Class Summary

These agents enhance bile salt-dependent biliary flow.

Ursodiol (Actigall, Urso)

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Shown to promote bile flow in cholestatic conditions associated with a patent extrahepatic biliary system. Following portoenterostomy in infants with biliary atresia, the drug may be useful in enhancing biliary drainage.

Class Summary

Long-term antibiotic prophylaxis may reduce the incidence of cholangitis following portoenterostomy.

Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

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Cholangitis is a common complication, both acutely and long term, following the Kasai procedure. When used prophylactically, may reduce the incidence of cholangitis, though conclusive supportive information is not available.

Questions

Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs. 2008 Dec. 17(12):1911-24. [QxMD MEDLINE Link].
Fischler B, Ehrnst A, Forsgren M, et al. The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr. 1998 Jul. 27(1):57-64. [QxMD MEDLINE Link].
Chang MH, Huang HH, Huang ES, et al. Polymerase chain reaction to detect human cytomegalovirus in livers of infants with neonatal hepatitis. Gastroenterology. 1992 Sep. 103(3):1022-5. [QxMD MEDLINE Link].
Wilson GA, Morrison LA, Fields BN. Association of the reovirus S1 gene with serotype 3-induced biliary atresia in mice. J Virol. 1994 Oct. 68(10):6458-65. [QxMD MEDLINE Link]. [Full Text].
Steele MI, Marshall CM, Lloyd RE, Randolph VE. Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease. Hepatology. 1995 Mar. 21(3):697-702. [QxMD MEDLINE Link].
Wang W, Donnelly B, Bondoc A, Mohanty SK, McNeal M, Ward R, et al. The rhesus rotavirus gene encoding VP4 is a major determinant in the pathogenesis of biliary atresia in newborn mice. J Virol. 2011 Jun 22. EPub ahead of print. [QxMD MEDLINE Link]. [Full Text].
Uemura M, Ozawa A, Nagata T, et al. Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice. Development. 2013 Feb. 140(3):639-48. [QxMD MEDLINE Link].
Superina R, Magee JC, Brandt ML, et al. The Anatomic Pattern of Biliary Atresia Identified at Time of Kasai Hepatoportoenterostomy and Early Postoperative Clearance of Jaundice Are Significant Predictors of Transplant-Free Survival. Ann Surg. 2011 Oct. 254(4):577-585. [QxMD MEDLINE Link].
[Guideline] Murray KF, Carithers RL Jr. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology. 2005 Jun. 41(6):1407-32. [QxMD MEDLINE Link].
Tessier ME, Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, et al. Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant. World J Gastroenterol. 2014 Aug 28. 20(32):11062-8. [QxMD MEDLINE Link]. [Full Text].
LeeVan E, Matsuoka L, Cao S, Groshen S, Alexopoulos S. Biliary-enteric drainage vs primary liver transplant initial treatment for children with biliary atresia. JAMA Surg. 2019 Jan 1. 154 (1):26-32. [QxMD MEDLINE Link].
Bassett MD, Murray KF. Biliary atresia: recent progress. J Clin Gastroenterol. 2008 Jul. 42(6):720-9. [QxMD MEDLINE Link].
Leung DH, Narang A, Minard CG, Hiremath G, Goss JA, Shepherd R. A 10-Year United Network for Organ Sharing review of mortality and risk factors in young children awaiting liver transplantation. Liver Transpl. 2016 Nov. 22 (11):1584-92. [QxMD MEDLINE Link].
Mogul D, Zhou M, Intihar P, Schwarz K, Frick K. Cost-effective analysis of screening for biliary atresia with the stool color card. J Pediatr Gastroenterol Nutr. 2015 Jan. 60 (1):91-8. [QxMD MEDLINE Link].
Harpavat S, Garcia-Prats JA, Anaya C, et al. Diagnostic yield of newborn screening for biliary atresia using direct or conjugated bilirubin measurements. JAMA. 2020 Mar 24. 323 (12):1141-50. [QxMD MEDLINE Link].
Ye W, Rosenthal P, Magee JC, Whitington PF, Childhood Liver Disease Research and Education Network. Factors Determining δ-Bilirubin Levels in Infants With Biliary Atresia. J Pediatr Gastroenterol Nutr. 2015 May. 60 (5):659-63. [QxMD MEDLINE Link].
Shneider BL, Magee JC, Karpen SJ, et al. Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia. J Pediatr. 2016 Mar. 170:211-217.e2. [QxMD MEDLINE Link].
Zhou L, Shan Q, Tian W, Wang Z, Liang J, Xie X. Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. AJR Am J Roentgenol. 2016 May. 206 (5):W73-82. [QxMD MEDLINE Link].
Shteyer E, Wengrower D, Benuri-Silbiger I, Gozal D, Wilschanski M, Goldin E. Endoscopic retrograde cholangiopancreatography in neonatal cholestasis. J Pediatr Gastroenterol Nutr. 2012 Aug. 55(2):142-5. [QxMD MEDLINE Link].
Ng VL, Haber BH, Magee JC, Miethke A, Murray KF, Michail S, et al. Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr. 2014 Sep. 165 (3):539-546.e2. [QxMD MEDLINE Link].
Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014 May 7. 311 (17):1750-9. [QxMD MEDLINE Link].
Chen Y, Nah SA, Chiang L, Krishnaswamy G, Low Y. Postoperative steroid therapy for biliary atresia: Systematic review and meta-analysis. J Pediatr Surg. 2015 Sep. 50 (9):1590-4. [QxMD MEDLINE Link].
Willot S, Uhlen S, Michaud L, Briand G, Bonnevalle M, Sfeir R, et al. Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics. 2008 Dec. 122(6):e1236-41. [QxMD MEDLINE Link].
Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009 Sep. 50(3):808-14. [QxMD MEDLINE Link]. [Full Text].
Barshes NR, Lee TC, Balkrishnan R, et al. Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transpl. 2005 Oct. 11(10):1193-200. [QxMD MEDLINE Link].
Bittmann S. Surgical experience in children with biliary atresia treated with portoenterostomy. Curr Surg. 2005 Jul-Aug. 62(4):439-43. [QxMD MEDLINE Link].
Chin LT, D'Alessandro AM, Knechtle SJ, et al. Liver transplantation for biliary atresia: 19-year, single-center experience. Exp Clin Transplant. 2004 Jun. 2(1):178-82. [QxMD MEDLINE Link].
Kasai M. Treatment of biliary atresia with special reference to hepatic porto- enterostomy and its modifications. Prog Pediatr Surg. 1974. 6:5-52. [QxMD MEDLINE Link].
Mack CL, Sokol RJ. Unraveling the pathogenesis and etiology of biliary atresia. Pediatr Res. 2005 May. 57(5 Pt 2):87R-94R. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Ryckman FC, Alonso MH, Bucuvalas JC, Balistreri WF. Biliary atresia--surgical management and treatment options as they relate to outcome. Liver Transpl Surg. 1998 Sep. 4(5 Suppl 1):S24-33. [QxMD MEDLINE Link].
[Guideline] Utterson EC, Shepherd RW, Sokol RJ, et al. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr. 2005 Aug. 147(2):180-5. [QxMD MEDLINE Link].
Visser BC, Suh I, Hirose S, et al. The influence of portoenterostomy on transplantation for biliary atresia. Liver Transpl. 2004 Oct. 10(10):1279-86. [QxMD MEDLINE Link].
Yoon PW, Bresee JS, Olney RS, et al. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997 Mar. 99(3):376-82. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Biliary atresia.
Bile ductular proliferation in liver biopsy specimen (hematoxylin and eosin stain) from patient with biliary atresia. Also note hepatocellular bile staining as a consequence of cholestasis.

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Author

Steven M Schwarz, MD, FAAP, FACN, AGAF Professor of Pediatrics, Children's Hospital at Downstate, State University of New York Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American Association for Physician Leadership, New York Academy of Medicine, Gastroenterology Research Group, American Gastroenterological Association, American Pediatric Society, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Stefano Guandalini, MD, AGAF Founder and Director Emeritus, Celiac Disease Center, University of Chicago; Former Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medicine; Professor Emeritus, The University of Chicago Pritzker School of Medicine

Stefano Guandalini, MD, AGAF is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology and Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, North American Society for the Study of Celiac Disease

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Imaware.health.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Pediatric Biliary Atresia Medication

Medication Summary

Bile acids

Class Summary

Ursodiol (Actigall, Urso)

Ursodiol (Actigall, Urso)

Antibiotics

Class Summary

Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

Pediatric Biliary Atresia Medication

Medication Summary

Bile acids

Class Summary

Ursodiol (Actigall, Urso)

Ursodiol (Actigall, Urso)

Antibiotics

Class Summary

Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

References

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