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Newborn Screening for Biliary Atresia: a Review of Current Methods

  • Pediatric Gastroenterology (S Orenstein and S Khan, Section Editors)
  • Published:
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Abstract

Purpose of Review

Biliary atresia is a serious neonatal liver disease due to obstructed bile ducts that has better outcomes when detected and treated in the first 30–45 days of life. This review examines different methods to screen newborns for biliary atresia as well as discusses observations from ongoing screening programs implemented in parts of the United States.

Recent Findings

Screening strategies for biliary atresia include detecting persistent jaundice, examining stool color, testing fractionated bilirubin levels, or measuring bile acid levels from dried blood spot cards. The stool color card program is the most widely used screening strategy worldwide. An alternative approach under investigation in the United States measures fractionated bilirubin levels, which are abnormal in newborns with biliary atresia. Fractionated bilirubin screening programs require laboratories to derive reference ranges, nurseries to implement universal testing, and healthcare systems to develop infrastructure that identifies and acts upon abnormal results.

Summary

Biliary atresia meets the disease-specific criteria for newborn screening. Current studies focus on developing a strategy which also meets all test-specific criteria. Such a strategy, if implemented uniformly, has the potential to accelerate treatment and reduce biliary atresia’s large liver transplant burden.

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Funding

SH is funded by NIH K23DK109207 and R03DK128535 as well as generous philanthropic support from Robert and Annie Graham. SLG is supported by the Intermountain Foundation at Primary Children’s Hospital.

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Authors and Affiliations

  1. Department of Pediatrics, University of Texas Health San Antonio, San Antonio, TX, USA

    Tebyan Rabbani

  2. Department of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Utah and Intermountain Primary Children’s Hospital, Salt Lake City, UT, USA

    Stephen L. Guthery

  3. Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Ochsner Health, New Orleans, LA, USA

    Ryan Himes

  4. Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children’s Hospital, 6621 Fannin Street, CCC 1010, Houston, TX, 77030, USA

    Benjamin L. Shneider & Sanjiv Harpavat

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  1. Tebyan Rabbani
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Correspondence to Sanjiv Harpavat.

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Conflicts of Interest/Competing Interests

SH is on a Data Safety Monitoring Board coordinated by Syneos Health, for a clinical trial that tests a therapy for biliary atresia.

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Rabbani, T., Guthery, S.L., Himes, R. et al. Newborn Screening for Biliary Atresia: a Review of Current Methods. Curr Gastroenterol Rep 23, 28 (2021). https://doi.org/10.1007/s11894-021-00825-2

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