Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes

Swati Antala; Sarah A Taylor

doi:10.1016/j.cld.2022.03.001

Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes

Clin Liver Dis. 2022 Aug;26(3):341-354. doi: 10.1016/j.cld.2022.03.001. Epub 2022 Jun 25.

Authors

Swati Antala¹, Sarah A Taylor²

Affiliations

¹ Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Ann and Robert H Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Box 65, Chicago, IL 60611, USA.
² Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Ann and Robert H Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Box 65, Chicago, IL 60611, USA. Electronic address: sataylor@luriechildrens.org.

Abstract

Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow progression after diagnosis. Variable contribution of genetic, immune, and environmental factors contributes to disease heterogeneity among patients with biliary atresia. Developing a deeper understanding of the disease mechanism will help to develop targeted medical therapies and improve patient outcomes.

Keywords: Biliary atresia; Neonatal cholestasis; Pediatric liver transplantation; Transplant outcomes.

Publication types

Review
Research Support, N.I.H., Extramural

MeSH terms

Biliary Atresia* / diagnosis
Biliary Atresia* / genetics
Biliary Atresia* / therapy
Child
Humans
Infant
Liver Transplantation*

Grants and funding

K08 DK121937/DK/NIDDK NIH HHS/United States