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Biliary Atresia Research Network Northeast

Recruiting
Conditions
Biliary Atresia
Registration Number
NCT06184971
Lead Sponsor
Yale University
Brief Summary

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.

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.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
150
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 & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Kasai procedure10 years

Need for biliary diversion procedure, including the Kasai portoenterostomy

Death10 years

Patient death

Liver transplant10 years

Need for liver transplant

Secondary Outcome Measures
NameTimeMethod
Clearance of jaundice6 months

Normalization of serum bilirubin after Kasai procedure

Bilirubin level10 years

Serum bilirubin level

Cholangitis10 years

Clinical diagnosis of cholangitis

Presentation to ED10 years

Patient presentation to the ED for complication related to biliary atresia

Age at follow-up with providers10 years

Frequency and age at most recent follow-up with Pediatric Surgeon and/or Hepatologist

Bleeding10 years

Post-operative bleeding requiring transfusion or return to the OR

Trial Locations

Locations (14)

Connecticut Children's Medical Center

🇺🇸

Hartford, Connecticut, United States

Yale University

🇺🇸

New Haven, Connecticut, United States

Eastern Maine Medical Center

🇺🇸

Bangor, Maine, United States

Maine Medical Center

🇺🇸

Portland, Maine, United States

Massachusetts General Hospital for Children

🇺🇸

Boston, Massachusetts, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Baystate

🇺🇸

Springfield, Massachusetts, United States

UMass Memorial Medical Center

🇺🇸

Worcester, Massachusetts, United States

Dartmouth-Hitchcock Medical Center

🇺🇸

Lebanon, New Hampshire, United States

Albany Medical Center/Bernard & Millie Duke's Children's Hospital

🇺🇸

Albany, New York, United States

John R. Oishei Children's Hospital

🇺🇸

Buffalo, New York, United States

University of Rochester Medical Center/Golisano Children's Hospital

🇺🇸

Rochester, New York, United States

SUNY Upstate Medical University

🇺🇸

Syracuse, New York, United States

Rhode Island Hospital/Hasbro Children's Hospital

🇺🇸

Providence, Rhode Island, United States

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