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Liver transplantation for congenital protein C deficiency with initial poor graft function: a case report with literature review

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Abstract

Congenital deficiency of protein C (PC) is a rare disease that causes thrombophilia during the neonatal and infantile periods. Despite anticoagulative treatments, purpura fulminans and major vessel thrombosis often occur. We report a 7-year-old girl with congenital PC deficiency who underwent deceased donor liver transplantation (LT) and experienced complications accompanied by initial poor graft function (IPGF). Before LT, she had cerebral and ophthalmic hemorrhage, and seven episodes of purpura fulminans. The operation was successfully performed; however, the liver graft developed IPGF. Hyperammonemia and coagulopathy required continuous hemodiafiltration and infusion of fresh frozen plasma. It took 22 days for PC activity to reach reference levels. The changes in clotting and anticlotting activities in the patient’s plasma were revealed using clot waveform analysis and the HemosIL ThromboPath® assay. PC activity remained normal for 5 years after LT. Even when IPGF occurs, liver function including PC activity can remain normal for a long time after recovery from IPGF. LT can be a curative treatment for congenital PC deficiency.

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Abbreviations

PC:

Protein C

LT:

Liver transplantation

IPGF:

Initial poor graft function

CWA:

Clot waveform analysis

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Acknowledgements

The authors wish to thank the medical editor from the Center for Postgraduate Education and Training at the NCCHD for editing this manuscript. This study was supported by AMED (20ek0109481h0001) and the NCCHD (29-8).

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Authors and Affiliations

  1. Center for Postgraduate Education and Training, National Center for Child Health and Development (NCCHD), Tokyo, Japan

    Atsushi Sakamoto & Akira Ishiguro

  2. Children’s Cancer Center, NCCHD, Tokyo, Japan

    Atsushi Sakamoto

  3. Division of Hematology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan

    Akira Ishiguro

  4. Organ Transplantation Center, NCCHD, Tokyo, Japan

    Akinari Fukuda, Seisuke Sakamoto & Mureo Kasahara

  5. Department of Pediatrics, Oita University, Oita, Japan

    So-ichi Suenobu

  6. Department of Pediatrics, Nara Medical University, Nara, Japan

    Tomoko Matsumoto & Keiji Nogami

  7. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

    Shouichi Ohga

Authors
  1. Atsushi Sakamoto
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  2. Akira Ishiguro
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  3. Akinari Fukuda
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  4. Seisuke Sakamoto
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  5. So-ichi Suenobu
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  6. Tomoko Matsumoto
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  7. Keiji Nogami
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  8. Shouichi Ohga
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  9. Mureo Kasahara
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Correspondence to Akira Ishiguro.

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Sakamoto, A., Ishiguro, A., Fukuda, A. et al. Liver transplantation for congenital protein C deficiency with initial poor graft function: a case report with literature review. Int J Hematol 114, 141–145 (2021). https://doi.org/10.1007/s12185-021-03103-z

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  • DOI: https://doi.org/10.1007/s12185-021-03103-z

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