Pediatric Biliary Atresia Medication

  • Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Oct 6, 2011
 

Medication Summary

In the immediate postoperative period, high-dose, pulse therapy with methylprednisolone has been used as both an anti-inflammatory agent and as a nonspecific stimulant of bile salt-independent bile flow. This therapy is currently being studied in a US government-sponsored clinical trial. In patients with chronic cholestatic conditions and bile duct patency, ursodeoxycholic acid (ie, ursodiol, UCDA) has also been shown to enhance bile flow.[8] For infants following portoenterostomy, UCDA may improve outcomes, and the drug is associated with minimal toxicity.

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.

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Bile acids

Class Summary

These agents enhance bile salt-dependent biliary flow.

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Ursodiol (Actigall, Urso)

 

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.

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Glucocorticoids

Class Summary

These agents elicit anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. Although no definitive data are available, methylprednisolone blast therapy (ie, high dose, short duration) has been used in the immediate postoperative period in an effort to establish bile drainage, in part by (theoretically) enhancing bile salt-independent bile flow.

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Methylprednisolone (Solu-Medrol)

 

Corticosteroids have long been recognized as potent anti-inflammatory agents and may stimulate bile salt-independent bile flow. Although their role in the postoperative management of biliary atresia has never been established, some centers have found that short-term, high-dose therapy in the immediate postoperative period may improve the likelihood of achieving adequate bile drainage, particularly for infants in whom bile flow is not immediately apparent following portoenterostomy.

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Antibiotics

Class Summary

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

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Trimethoprim-Sulfamethoxazole (Bactrim, Septra)

 

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.

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Contributor Information and Disclosures
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 College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Specialty Editor Board

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, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

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  Director, Celiac Disease Center, Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medical Center; Professor, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

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

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

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, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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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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