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Developmental histology of the portal plate in biliary atresia: observations and implications

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Abstract

Purpose

The key characteristic of biliary atresia (BA) is obliteration of the extrahepatic bile ducts at the level of the porta hepatis. We aimed to relate the immunohistochemical features of remnant biliary ductules at the porta hepatis with clinical features and outcomes.

Methods

Samples were immunostained with anti-cytokeratin 20 (CK20), vimentin and alpha-smooth muscle actin (aSMA). Primary outcome was set as clearance of jaundice (bilirubin ≤ 20 μmol/L) following Kasai portoenterostomy (KPE).

Results

Eighty-two cases were classified into syndromic BA (n = 10), cystic BA (n = 7), CMV IgM+ BA (n = 9) and isolated BA (n = 56). CK20 expression was confirmed in 40/82 (49%), and vimentin expression in 19/82 (23%). aSMA was negative in all cases studied. CK20 expression was less common in isolated BA (n = 20/56, 36%) compared to CMV IgM+ BA (n = 8/9, 89%), cystic BA (n = 7/7, 100%) (isolated BA vs non-isolated BA, P = 0.0008). There was no difference in vimentin expression among the sub-groups (isolated BA vs. non-isolated BA; P = 0.39). CoJ was achieved in 52/82 (63%) overall with significant difference depending simply on sub-group [e.g. syndromic BA 9/10 (90%)]. CK20 expression was associated with a diminished rate of CoJ in the entire cohort [CK20+ 32/56 (57%) vs. CK20− 20/26 (77%); P = 0.04]. By contrast no correlation was observed between vimentin expression and CoJ (P = 0.13).

Conclusion

CK20+ expression was associated with reduced clearance of jaundice in BA and a trend towards reduced native liver survival.

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Acknowledgements

We recognise the superb technical skill and help with processing histological material at the laboratories of the Institute of Liver Studies.

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Authors and Affiliations

  1. Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London, SE5 9RS, UK

    Enrico La Pergola & Mark Davenport

  2. Institute of Liver Studies, Kings College Hospital, Denmark Hill, London, SE5 9RS, UK

    Yoh Zen

  3. Department of Pediatric Surgery, Università degli Studi di Padova, Padua, Italy

    Enrico La Pergola

Authors
  1. Enrico La Pergola
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  2. Yoh Zen
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  3. Mark Davenport
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Contributions

All authors contributed to the study conception and design. Laboratory work was performed by ELP and YZ. Data analysis were performed by MD. The first draft of the manuscript was written by MD and YZ and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Mark Davenport.

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La Pergola, E., Zen, Y. & Davenport, M. Developmental histology of the portal plate in biliary atresia: observations and implications. Pediatr Surg Int 37, 715–721 (2021). https://doi.org/10.1007/s00383-021-04861-x

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  • DOI: https://doi.org/10.1007/s00383-021-04861-x

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