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Budd-Chiari Syndrome Due to Protein C Deficiency: A Case Report
1About the authors: Lovely Negi, MSc, MSN, RN, is a Lecturer, College of Nursing, Institute of Liver and Biliary Sciences, New Delhi.
Summary
Budd-Chiari Syndrome (BCS), a rare liver vascular disorder, was linked to protein C deficiency in a patient with an inferior vena cava thrombus. Early recognition and multidisciplinary care are crucial for managing this uncommon association.
Area of Science:
- Hepatology
- Vascular Medicine
- Hematology
Background:
- Budd-Chiari Syndrome (BCS) involves hepatic venous outflow obstruction, affecting approximately 1 in 100,000 people.
- Coagulation factor deficiencies are an infrequent cause of BCS.
- Protein C deficiency is a rare inherited thrombophilia.
Purpose of the Study:
- To report a rare case of BCS associated with protein C deficiency.
- To highlight the diagnostic and management challenges in such cases.
- To emphasize the importance of early intervention and multidisciplinary care.
Main Methods:
- Case report adhering to CARE guidelines.
- Clinical presentation, laboratory investigations, and imaging studies (ultrasound, CT/MRI) were used for diagnosis.
- Treatment involved interventional angioplasty, anticoagulation, and diuretics.
Main Results:
- A 5.2 cm thrombus in the inferior vena cava and complete fibrotic occlusion of hepatic veins were identified.
- The patient presented with pedal edema and abdominal distension.
- Successful angioplasty and medical management were initiated.
Conclusions:
- This case underscores the rare association between protein C deficiency and BCS.
- Early diagnosis and comprehensive management, including potential liver transplantation, are vital.
- Multidisciplinary care and supportive nursing are essential for improving patient outcomes.
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