Biliary Atresia Research Network Northeast (BARNN)

Biliary Atresia Research Network Northeast (BARNN)

This is a multi-center retrospective chart review to compile a data repository of the management and outcomes of children with biliary atresia. Overall, investigators aim to evaluate which specific factors contribute to improved patient outcomes, to help guide potential improvements in patient care and resource utilization.

Study Overview

• Status: Active, not recruiting
• Conditions: Biliary Atresia

Detailed Description

Biliary atresia is quite rare, with only 0.73 cases per 10,000 births in the US. Due to this rarity and the geographical spread of the US, the small number of cases end up being dispersed amongst the various pediatric hospitals, which leads to certain hospitals only encountering an affected patient once per decade. This rarity and dispersion makes biliary atresia difficult for researchers to study: single-institution studies are limited by low power and only provide narrow snapshots, whereas large NIH-sponsored consortia report highly selected outcomes from only the largest or most dedicated centers and have largely excluded New England. This leaves a significant knowledge gap regarding the management and outcomes at more typical hospitals. The purpose of this study is to collect clinical data from all children with biliary atresia at all hospitals providing pediatric surgical care in the Northeast, even very-low-volume hospitals. This is a retrospective study, only involving chart review. There will be no interaction with subjects, intervention, or collection of specimens for the purposes of this study. The data will only include clinical information that was recorded during the normal course of patient care. The subjects will be de-identified before entry into a HIPAA-compliant data repository. This data repository will allow researchers to pool data, to yield adequate statistical power and assess differences in management and outcomes regarding this very rare condition.

• Study Type: Observational
• Enrollment (Estimated): 150

Contacts and Locations

Study Locations

• United States
  • Connecticut
    • Hartford, Connecticut, United States, 06106
      • Connecticut Children's Medical Center
    • New Haven, Connecticut, United States, 06510
      • Yale University
  • Maine
    • Bangor, Maine, United States, 04401
      • Eastern Maine Medical Center
    • Portland, Maine, United States, 04102
      • Maine Medical Center
  • Massachusetts
    • Boston, Massachusetts, United States, 02115
      • Boston Children's Hospital
    • Boston, Massachusetts, United States, 02114
      • Massachusetts General Hospital for Children
    • Springfield, Massachusetts, United States, 01199
      • Baystate
    • Worcester, Massachusetts, United States, 01605
      • UMass Memorial Medical Center
  • New Hampshire
    • Lebanon, New Hampshire, United States, 03756
      • Dartmouth-Hitchcock Medical Center
  • New York
    • Albany, New York, United States, 12208
      • Albany Medical Center/Bernard & Millie Duke's Children's Hospital
    • Buffalo, New York, United States, 14203
      • John R. Oishei Children's Hospital
    • Rochester, New York, United States, 14642
      • University of Rochester Medical Center/Golisano Children's Hospital
    • Syracuse, New York, United States, 13210
      • SUNY Upstate Medical University
  • Rhode Island
    • Providence, Rhode Island, United States, 02905
      • Rhode Island Hospital/Hasbro Children's Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

Children 12 years and younger (at the time of the qualifying encounter for care) who were diagnosed with biliary atresia, underwent surgical intervention for biliary atresia, and/or had an encounter for care related to biliary atresia at a participating institution during the study period. Patients who did not undergo surgery will be included (e.g. patients who died before surgery).

Description

Inclusion Criteria:

• Children with a healthcare encounter related to biliary atresia at a participating institution during the study period (January 1st, 2012 through December 31st, 2021)
• Age 12 years and younger at the time of the qualifying encounter during the study period (encompassing patients born between January 2nd, 1999 through December 31st, 2021)

• Diagnosis of biliary atresia, based on one of the following ICD-10 codes:

  • Q44.2 Atresia of bile ducts
  • Q44.3 Congenital stenosis and stricture of bile ducts
  • Q44.4 Disorders of the biliary tract with major complication or comorbidity
  • Q44.5 Other congenital malformations of the bile ducts
  • Q44.6 Disorders of the biliary tract without major complication or comorbidity

Exclusion Criteria:

• Patients aged 13 years old or older during the study period (i.e. those born before January 2nd, 1999)
• Patients born and/or diagnosed with biliary atresia after the end of the study period (i.e. those born after December 31st, 2021)
• No diagnosis of biliary atresia
• History of biliary atresia without any episodes of care related to biliary atresia during the study period (e.g. a teenager with history of BA who underwent Kasai procedure as an infant and has no current issues related to their BA and is receiving care for an unrelated reason)

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Death	Patient death	10 years
Kasai procedure	Need for biliary diversion procedure, including the Kasai portoenterostomy	10 years
Liver transplant	Need for liver transplant	10 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clearance of jaundice	Normalization of serum bilirubin after Kasai procedure	6 months
Bilirubin level	Serum bilirubin level	10 years
Cholangitis	Clinical diagnosis of cholangitis	10 years
Bleeding	Post-operative bleeding requiring transfusion or return to the OR	10 years
Presentation to ED	Patient presentation to the ED for complication related to biliary atresia	10 years
Age at follow-up with providers	Frequency and age at most recent follow-up with Pediatric Surgeon and/or Hepatologist	10 years

Collaborators and Investigators

• Sponsor: Yale University
• Investigators: Principal Investigator: Robert Cowles, MD, Yale University

Study record dates

Study Major Dates

• Study Start (Actual): May 6, 2024
• Primary Completion (Estimated): May 1, 2026
• Study Completion (Estimated): December 1, 2026

Study Registration Dates

• First Submitted: December 13, 2023
• First Submitted That Met QC Criteria: December 13, 2023
• First Posted (Actual): December 29, 2023

Study Record Updates

• Last Update Posted (Actual): April 2, 2026
• Last Update Submitted That Met QC Criteria: March 27, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Digestive System Diseases; Biliary Tract Diseases; Congenital Abnormalities; Bile Duct Diseases; Digestive System Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Biliary Atresia
• Other Study ID Numbers: 2000035645; 000 (Other Identifier: YCTG)

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No