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Liver fibrosis in biliary atresia

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Abstract

Background

Biliary atresia (BA) is the most common cause of obstructive jaundice in infants. Although the Kasai procedure has greatly improved the prognosis, most patients still need liver transplantation (LT) for long-term survival. The pathogenesis of BA has not been fully clarified, and liver fibrosis in BA is far beyond biliary obstructive cirrhosis.

Data sources

Literature reviews were underwent through PubMed. Persistent inflammation, immune response, biliary epithelial–mesenchymal transition, matrix deposition, decompensated angiogenesis, and unique biliary structure development all contribute to the fibrosis process. Observed evidences in such fields have been collected and form the backbone of this review.

Results

Interactions of the multiple pathways accelerate this process.

Conclusions

Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.

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Funding

This work was supported by the Natural Science Foundation of China (No. 81400576).

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Authors and Affiliations

  1. Department of Pediatric Surgery, Children’s Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China

    Wen-Jun Shen, Gong Chen, Min Wang & Shan Zheng

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  1. Wen-Jun Shen
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  2. Gong Chen
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  3. Min Wang
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  4. Shan Zheng
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Contributions

WJS and MW reviewed and wrote the draft, and SZ and GC directed and edited this scientific work. All the authors approved the final version of the manuscript.

Corresponding author

Correspondence to Shan Zheng.

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Ethical approval

This work had the permit from IRB of the Children’s Hospital of Fudan University.

Conflict of interest

The authors have no conflicts of interest or ties to disclose.

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Shen, WJ., Chen, G., Wang, M. et al. Liver fibrosis in biliary atresia. World J Pediatr 15, 117–123 (2019). https://doi.org/10.1007/s12519-018-0203-1

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