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Epidemiological Characteristics and Etiology of Budd-Chiari Syndrome in Upper Egypt
Muhamad R Abdel Hameed1, Esam Abdel-Moneim Sadek Elbeih1, Heba Mahmoud Abd El-Aziz2
1Department of Internal Medicine & Hematology Unit, Assiut University Hospitals and Bone Marrow Transplantation Unit, South Egypt Cancer Institute, Assiut University, Assiut, Egypt.
Insights
Budd-Chiari syndrome (BCS) in Upper Egypt predominantly affects males in their third and fourth decades, often presenting with liver cirrhosis. Protein C deficiency is the most frequent cause, followed by Factor V Leiden mutation and protein S deficiency.
Area of Science:
- Hepatology
- Vascular Medicine
- Genetics
Background:
- Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction.
- It disproportionately affects developing countries and typically manifests in the third or fourth decade of life.
Purpose of the Study:
- To elucidate the sociodemographic characteristics, clinical presentations, radiological findings, and etiologies of BCS in Upper Egyptian patients.
- To identify specific risk factors and etiological agents contributing to BCS in this population.
Main Methods:
- A retrospective cohort study involving 50 Upper Egyptian patients with confirmed primary BCS.
- Comprehensive evaluation included liver function tests, coagulation profiles, thrombophilia screening (anticardiolipin antibodies, lupus anticoagulant, protein C, protein S, antithrombin III), Factor V Leiden and JAK2 mutation analysis, and full radiological assessment.
Main Results:
- The study included 50 patients (56% male, 44% female) with a mean age of 32.5 years.
- Identified etiologies included isolated protein C deficiency (26%), Factor V Leiden mutation (10%), isolated protein S deficiency (10%), antiphospholipid syndrome (8%), and membranous web (8%). Etiology remained unidentified in 22% of cases.
- The majority of patients (82%) had liver cirrhosis (Child class C in 66%), with abdominal pain (96%) and ascites (82%) being the most common symptoms and signs. Hepatic vein obstruction was observed in 80% of cases.
Conclusions:
- BCS in Upper Egypt primarily affects males in their third and fourth decades, frequently associated with liver cirrhosis.
- The leading causes are isolated protein C deficiency, Factor V Leiden mutation, and isolated protein S deficiency.
- Hepatic vein obstruction represents the most common vascular involvement pattern in this cohort.
Background And Objectives:
Budd-Chiari syndrome (BCS) is a rare disorder caused by obstruction to hepatic venous outflow. It affects all races, usually during the third or fourth decade of life. Higher prevalence had being evident in developing countries. The aim of the present study was to clarify sociodemographic features, clinical, radiological presentations, and etiology of BCS among Upper Egyptian patients.
Patients And Methods:
This retrospective cohort study enrolled 50 Upper Egyptian Patients with confirmed primary BCS. Liver, coagulation, and thrombophilia workup profiles were performed as anticardiolipin antibodies, lupus anticoagulant, protein C, protein S, and antithrombin III assays. Factor V Leiden and JAK2 mutations were assessed. Full radiological assessment was done.
Results:
Fifty patients were included. There were 28 males (56%) and 22 females (44%) with mean age (32.5 ± 11.1 years). The etiological factor was not identified in 22% of cases (n=11). Isolated factor C deficiency was found in 26% (n=13) with male predominance 39.3% and protein S deficiency in 10% (n=5). Factor V Leiden mutation was the etiology in 5 patients (10%). Membranous web and antiphospholipid syndrome each were the etiology in 8% (n=4). Behςet's disease was diagnosed in 4% (n=2). Cases of liver cirrhosis(LC) were 41/50(82%)they were :33/50(66%) LC child class C, 8 /50(16%) LC child class B, and 0/50 (0%) LC child class A. Abdominal pain was the most common symptom (96%), and ascites was the most common sign (82%). Obstruction of hepatic veins was present in 80%.
Conclusion:
BCS in Upper Egyptian patients was mainly occurred in males in the third and fourth decade of life, mostly with liver cirrhosis. The most common etiology is isolated protein C deficiency followed by Factor V Leiden mutation and isolated protein S deficiency. Hepatic veins obstruction was the most common pattern of vascular involvement.
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