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Budd-Chiari syndrome attributed to protein C deficiency
1Department of Internal Medicine, Gastroenterology/Hepatology Division, Saiseikai Wakakusa Hospital, Yokohama, Japan.
Insights
Protein C deficiency, a condition affecting blood coagulation, may cause Budd-Chiari syndrome. This case study highlights a patient with significantly reduced protein C levels and inferior vena cava occlusion.
Area of Science:
- Biochemistry
- Hematology
- Vascular Medicine
Background:
- The protein C anticoagulant pathway plays a crucial role in regulating the blood coagulation cascade.
- Budd-Chiari syndrome, characterized by hepatic venous outflow obstruction, can lead to severe liver damage.
Abstract:
The protein C anticoagulant pathway is an important downregulating mechanism of the blood coagulation cascade. We report a 47-yr-old male with Budd-Chiari syndrome and occlusion of the inferior vena cava. His plasma protein C antigen level was decreased to 42%, and its activity was 34%. Other coagulable factors were normal. Protein C deficiency should be considered a possible etiological factor of the Budd-Chiari syndrome.