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

Atsushi Sakamoto, Akira Ishiguro, Akinari Fukuda, Seisuke Sakamoto, So ichi Suenobu, Tomoko Matsumoto, Keiji Nogami, Shouichi Ohga, Mureo Kasahara

研究成果: Contribution to journalArticle査読

抄録

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.

本文言語英語
ジャーナルInternational journal of hematology
DOI
出版ステータス受理済み/印刷中 - 2021

All Science Journal Classification (ASJC) codes

  • Hematology

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