TY - JOUR
T1 - Liver transplantation for congenital protein C deficiency with initial poor graft function
T2 - a case report with literature review
AU - Sakamoto, Atsushi
AU - Ishiguro, Akira
AU - Fukuda, Akinari
AU - Sakamoto, Seisuke
AU - Suenobu, So ichi
AU - Matsumoto, Tomoko
AU - Nogami, Keiji
AU - Ohga, Shouichi
AU - Kasahara, Mureo
N1 - Funding Information:
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).
Funding Information:
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).
Publisher Copyright:
© 2021, Japanese Society of Hematology.
PY - 2021/7
Y1 - 2021/7
N2 - 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.
AB - 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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U2 - 10.1007/s12185-021-03103-z
DO - 10.1007/s12185-021-03103-z
M3 - Article
C2 - 33638034
AN - SCOPUS:85101610057
VL - 114
SP - 141
EP - 145
JO - International Journal of Hematology
JF - International Journal of Hematology
SN - 0925-5710
IS - 1
ER -