Long‐Term Restoration of Anticoagulant Function After Liver Transplantation for Severe Congenital Protein C Deficiency: A Single‐Center Experience Involving Three Pediatric Patients

Severe congenital protein C (PC) deficiency is a rare inherited coagulation disorder that can be difficult to control with conventional medical treatment alone. We report a single-center case series of three pediatric patients with severe congenital PC deficiency who underwent liver transplantation (LT). In all patients, plasma PC activity levels exceeded 64% after LT and remained stable without anticoagulation treatment or PC supplementation. Two patients were followed for more than 10 years, representing the longest reported outcomes to date. Stable anticoagulant function persisted despite rejection, graft dysfunction, and subsequent surgery, demonstrating durable restoration of physiological anticoagulant function after LT.

Authors

Institutions

Publication Details

Journal
Pediatric Blood & Cancer
Published
2026-08-27
DOI
https://doi.org/10.1002/1545-5017.70646
Primary Topic
Blood Coagulation and Thrombosis Mechanisms
Type
article
Field-Weighted Citation Impact
0.00

Funders

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Long‐Term Restoration of Anticoagulant Function After Liver Transplantation for Severe Congenital Protein C Deficiency: A Single‐Center Experience Involving Three Pediatric Patients

Akira Ishiguro, Mureo Kasahara, Akinari Fukuda, Atsushi Sakamoto et al.
Pediatric Blood & Cancer
Blood Coagulation and Thrombosis Mechanisms
article

Long‐Term Restoration of Anticoagulant Function After Liver Transplantation for Severe Congenital Protein C Deficiency: A Single‐Center Experience Involving Three Pediatric Patients

Akira Ishiguro, Mureo Kasahara, Akinari Fukuda, Atsushi Sakamoto, Shouichi Ohga, Seisuke Sakamoto, Kotaro Sakaya
article en

Abstract

Severe congenital protein C (PC) deficiency is a rare inherited coagulation disorder that can be difficult to control with conventional medical treatment alone. We report a single-center case series of three pediatric patients with severe congenital PC deficiency who underwent liver transplantation (LT). In all patients, plasma PC activity levels exceeded 64% after LT and remained stable without anticoagulation treatment or PC supplementation. Two patients were followed for more than 10 years, representing the longest reported outcomes to date. Stable anticoagulant function persisted despite rejection, graft dysfunction, and subsequent surgery, demonstrating durable restoration of physiological anticoagulant function after LT.

Pediatric Blood & Cancer
Kyushu University (JP), National Center For Child Health and Development (JP), Japan Clinical Cancer Research Organization (JP), Organ Technologies (Japan) (JP), National Disaster Medical Center (JP)
National Center for Child Health and Development, Japan Agency for Medical Research and Development
Openalex Percentile: Top 11%
Blood Coagulation and Thrombosis Mechanisms
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.