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Biliary Atresia as a Disease Starting In Utero

Implications for Treatment, Diagnosis, and Pathogenesis

Mysore, Krupa R.; Shneider, Benjamin L.; Harpavat, Sanjiv

Journal of Pediatric Gastroenterology and Nutrition: October 2019 - Volume 69 - Issue 4 - p 396–403
doi: 10.1097/MPG.0000000000002450
Review Article
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ABSTRACT Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.

Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children's Hospital, Houston, TX.

Address correspondence and reprint requests to Sanjiv Harpavat, MD, PhD, Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children's Hospital, 6621 Fannin St, CCC 1010, Houston, TX 77030 (e-mail: harpavat@bcm.edu).

Received 10 January, 2019

Accepted 23 June, 2019

Funding for this study came from NIH 1 K23 DK109207 (S.H.) and the Cade R. Alpard Foundation for Pediatric Liver Disease.

The authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,