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Positive C4d staining of the portal vein endothelium in the liver of patients with biliary atresia: a role of humoral immunity in ongoing liver fibrosis

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Abstract

Purpose

This study aimed to clarify the role of complement activation in fibrogenesis in BA.

Methods

In total, 27 paraffin-embedded liver biopsy samples were immunohistochemically analyzed using C4d polyclonal antibody, vascular cell adhesion molecule-1 (VCAM-1), and CD45. The liver samples were obtained from 25 patients during Kasai operation, and two additional specimens were obtained from 2 patients by needle biopsy later at the time of liver function deterioration. The degree of liver fibrosis was histologically graded 1–3.

Results

Among the 25 samples, 9 showed C4d-positive immunostaining localized on the endothelia of a few portal veins in the portal tract. The degree of fibrosis was correlated with C4d staining (p = 0.025). The age at Kasai operation correlated with the degree of fibrosis and the C4d positivity. Two needle biopsy samples were positive for C4d. Among 13 samples submitted for VCAM-1 staining, 2 negative samples were C4d negative and all positive C4d samples were VCAM-1 positive with CD45 mononuclear cell infiltration.

Conclusion

These findings suggest that ongoing cirrhosis could be a result of progressive “vasculopathy” of the portal vein caused by humoral and cell-mediated immune interaction.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Ibaraki Children’s Hospital, 3-3-1 Futabadai, Mito, Ibaraki, 311-4145, Japan

    Sorahiko Fujisawa, Toshihiro Muraji, Naoya Sakamoto, Satoshi Matsuda, Hajime Kawakami, Misako Hirai & Toshihiro Yanai

  2. Department of Pathology, Kori Hospital, Kansai Medical University, 8-45 Korihondori, Neyagawa, Osaka, 572-8551, Japan

    Naoki Hosaka

Authors
  1. Sorahiko Fujisawa
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  2. Toshihiro Muraji
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  3. Naoya Sakamoto
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  4. Naoki Hosaka
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  5. Satoshi Matsuda
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  6. Hajime Kawakami
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  7. Misako Hirai
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  8. Toshihiro Yanai
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Corresponding author

Correspondence to Toshihiro Muraji.

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Fujisawa, S., Muraji, T., Sakamoto, N. et al. Positive C4d staining of the portal vein endothelium in the liver of patients with biliary atresia: a role of humoral immunity in ongoing liver fibrosis. Pediatr Surg Int 30, 877–881 (2014). https://doi.org/10.1007/s00383-014-3553-3

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  • DOI: https://doi.org/10.1007/s00383-014-3553-3

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