• The percentage of patients with serum total bilirubin <1.5 mg/dL and with native liver at 6 months after portoenterostomy [ Time Frame: Measurements will be made at 12 and 24 months of age ] [ Designated as safety issue: No ]
  

• Survival with native liver at 24 months of age [ Time Frame: Measurements will be made at 12 and 24 months of age ] [ Designated as safety issue: No ]
  
• Serum total bilirubin concentration [ Time Frame: Measurements will be made at 3 months after portoenterostomy and at 12 and 24 months of age ] [ Designated as safety issue: No ]
  
• Growth [ Time Frame: Measurements will be made at 12 and 24 months of age ] [ Designated as safety issue: No ]
  
• Serum biomarkers of sufficiency of fat-soluble vitamins [ Time Frame: Measurements will be made at 12 and 24 months of age ] [ Designated as safety issue: No ]
  
• Presence of ascites [ Time Frame: Measurements will be made at 12 and 24 months of age ] [ Designated as safety issue: No ]
  

This is a multi-center randomized, double-blinded, placebo-controlled trial to prospectively determine the efficacy of corticosteroids on the outcome of infants with biliary atresia. The trial will be conducted by the NIDDK-funded network of ten clinical centers comprising the Biliary Atresia Clinical Research Consortium (BARC), whose goal is to study the etiology, pathogenesis, diagnosis, and treatment of infants with biliary atresia. For the trial, our overall hypothesis is that therapy with corticosteroids following portoenterostomy (including gall bladder Kasai procedure) will improve bile drainage and long-term outcome in infants with biliary atresia. This hypothesis will be tested through the following specific aims and hypotheses:

Aim 1: To determine whether corticosteroid therapy decreases serum bilirubin concentration after portoenterostomy.

Aim 2: To determine whether corticosteroid treatment after portoenterostomy will improve outcome as defined by survival without transplantation at 24 months of age.

Aim 3: To determine whether corticosteroid treatment after portoenterostomy will improve growth of infants with biliary atresia.

Aim 4: To determine whether corticosteroid treatment improves biochemical indicators of each of the fat-soluble vitamins after supplementation with standard doses.

Aim 5: To determine whether corticosteroid treatment after portoenterostomy will decrease the incidence of persistent ascites or ascites that requires medical treatment.

The significance of the proposed trial is that it will determine whether corticosteroids are an effective medical treatment to improve bile drainage and long-term outcome, and whether its use reduces the need for liver transplantation in infants with biliary atresia.

Subjects will be recruited from patients enrolled in the BARC prospective observational database study who undergo portoenterostomy or portochelecystostomy (gall bladder Kasai) for biliary atresia.

The Primary outcome measure is the percentage of patients with serum total bilirubin <1.5 mg/dL and with native liver at 6 months after portoenterostomy.

Secondary outcome measures are:

1. Serum total bilirubin concentration (and also at 3 months after portoenterostomy)
2. Survival with native liver at 24 months of age

3. Growth

   1. Weight for age Z-score (in patients without ascites)
   2. Height for age Z score

4. Serum biomarkers of sufficiency of fat-soluble vitamins

   1. Vitamin A: molar ratio of serum retinol/retinol binding protein
   2. Vitamin D: serum level of 25-hydroxy vitamin D
   3. Vitamin E: ratio of serum vitamin E/total lipids
   4. Vitamin K: International Normalized Ratio (INR)
5. Presence of ascites

All measurements will be made at 12 and 24 months of age (unless noted otherwise):

Inclusion Criteria:

• Portoenterostomy or gall bladder Kasai operation for biliary atresia within the previous 72 hours
• Post-conception age ≥ 36 weeks
• Weight at enrolment ≥ 2000 gm
• Written informed consent to participate in the study obtained prior to or within 72 hours of completion of portoenterostomy. (Note: Families of potential subjects may be approached prior to the portoenterostomy.)

Exclusion Criteria:

• Known immunodeficiency
• Diabetes mellitus
• Presence of significant systemic hypertension for age (persistent systolic blood pressure ≥112 mmHg)
• A serum indirect (unconjugated) bilirubin ≥ 5 mg/dL for infants under 4 weeks of age or ≥ 7 mg/dL for infants between 4 and 8 weeks of age
• Known sensitivity to corticosteroids
• Documented bacteremia or other tissue infection which is felt to be clinically relevant
• Known congenital infection or disease with herpes simplex virus, toxoplasmosis, or cytomegalovirus inclusion disease of the liver
• Infants whose mother is known to have human immunodeficiency virus infection
• Infants whose mother is known to be HBsAg or hepatitis C virus positive
• Infants with other severe concurrent illnesses such as neurological, cardiovascular, pulmonary, metabolic, endocrine, and renal disorders that would interfere with the conduct and results of the study
• Any other clinical condition that is a contraindication to the use of corticosteroid (e.g., bowel perforation)