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Published on: September 9, 2012
Protein C and protein S deficiency presenting as Budd-Chiari syndrome
Periasamy Uvaraj1, Ramar Rathisharmila, Veerappan Ilamaran
1Melmaruvathur Adhiparasakthi Institute of Medical Sciences and Research, Tamilnadu, India. dr_uvaraj@yahoo.co.in
Insights
Combined deficiencies in natural anticoagulants Protein C and Protein S, though rare, significantly increase thrombotic risk. This case highlights a pediatric Budd-Chiari syndrome linked to this dual deficiency, managed with anticoagulation.
Area of Science:
- Hematology
- Pediatric Thrombosis
- Coagulation Disorders
Background:
- Protein C and Protein S are crucial vitamin K-dependent anticoagulants regulating hemostasis.
- Deficiencies in these proteins elevate the risk of developing thrombotic events.
- Combined Protein C and S deficiency is an exceptionally rare clinical finding.
Abstract:
Protein C and S are vitamin K-dependent natural anticoagulants. They play a major role in hemostasis by degrading the activated factor V and factor VIII. Deficiencies of protein C and protein S are associated with increased risk of thrombotic events. The combined occurrence of protein C and S deficiency has been rarely reported. We report a 6-year-old boy with Budd-Chiari syndrome due to combined protein C and protein S deficiency. He was managed with low molecular weight heparin and discharged on long-term warfarin therapy.
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