Long-Term Restoration of Anticoagulant Function After Liver Transplantation for Severe Congenital Protein C Deficiency: A Single-Center Experience Involving Three Pediatric Patients.
Kotaro Sakaya, Akira Ishiguro, Atsushi Sakamoto et al.
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Case in brief
Three pediatric patients with severe congenital protein C deficiency underwent liver transplantation, resulting in plasma protein C activity levels exceeding 64% post-surgery. Remarkably, these levels remained stable without the need for anticoagulation treatment or protein C supplementation. Two patients were followed for over 10 years, showcasing long-term restoration of anticoagulant function despite challenges such as rejection and graft dysfunction.
Diagnostic / clinical pearl
Liver transplantation can provide durable restoration of anticoagulant function in pediatric patients with severe congenital protein C deficiency, even in the presence of graft complications.
Why it matters
This case series highlights the potential of liver transplantation as a viable treatment option for managing severe congenital protein C deficiency, offering long-term benefits beyond conventional therapies.