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

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

Swati Antala  1 , Sarah A Taylor  2
Affiliations
Review

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

Swati Antala et al. Clin Liver Dis. 2022 Aug.

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.

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Conflict of interest statement

Disclosure The authors report no conflict of interest.

Figures

Figure 1.
Figure 1.
Liver biopsy from an infant with BA at diagnosis that underwent liver transplant before 2 years of age. Histologic findings include expansion of the portal tract with stromal edema, prominent fibrosis, bile duct proliferation (black arrows), and bile plugs (white arrows). White star delineates a branching artery.
Figure 2.
Figure 2.
A. Early immune mechanism of BA is characterized by activation of innate immunity via PAMPs and DAMPs with subsequent stimulation of the adaptive immune response. B. Later stages of BA exhibit a Th2/Th17 phenotype with IL-33/IL-13 driven bile duct proliferation and fibrosis. CCL2 – C-C motif chemokine ligand 2; DAMP - damage associated molecular pattern; DC – dendritic cell; ECM – extracellular matrix; IFN – interferon; IL – interleukin; NK – natural killer cell; PAMP - pathogen associated molecular pattern; TLR – toll-like receptor; TNF - tumor necrosis factor; Tregs - T regulatory cells
Figure 3.
Figure 3.
Cumulative data on the overall prognosis of BA demonstrates about 50% of patients require liver transplantation by 2 years of age. CMV – cytomegalovirus; KPE - Kasai portoenterostomy
See this image and copyright information in PMC

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