title: Epidemiological aspects of Budd-Chiari in Egyptian patients: a single-center study.
abstract: OBJECTIVE
  To describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome (BCS) in Egyptian patients.
  
  
  METHODS
  Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group (BCSG) and admitted to the Tropical Medicine Department of Ain Shams University Hospital (Cairo, Egypt). Complete clinical evaluation and laboratory investigations, including a thrombophilia workup and full radiological assessment, were performed to determine underlying disease etiologies.
  
  
  RESULTS
  BCS was chronic in 79.8% of patients, acute or subacute in 19.1%, and fulminant in 1.1%. Factor V Leiden mutation (FVLM) was the most common etiological cause of disease (53.1%), followed by mutation of the gene encoding methylene tetrahydrofolate reductase (MTHFR) (51.6%). Current or recent hormonal treatment was documented in 15.5% of females, and BCS associated with pregnancy was present in 17.2% of females. Etiology could not be determined in 8.5% of patients. Males had significantly higher rates of MTHFR gene mutation and Behçet's disease, and females had significantly higher rates of secondary antiphospholipid antibody syndrome. A highly significant positive relationship was evident between the presence of Behçet's disease and inferior vena caval occlusion, either alone or combined with occlusion of the hepatic veins (P < 0.0001).
  
  
  CONCLUSIONS
  FVLM is the most common disease etiology and MTHFR the second most common in Egyptian BCS patients. BCS etiology tends to vary with geographic region.
authors.affiliation: Department of Tropical Medicine, Faculty of Medicine, Ain Shams University, Abbassia, Cairo 11341, Egypt.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Sakr
authors.forename: Eman
authors.initials: E
authors.lastname: Barakat
authors.forename: Sara
authors.initials: S
authors.lastname: Abdelhakam
authors.forename: Hany
authors.initials: H
authors.lastname: Dabbous
authors.forename: Said
authors.initials: S
authors.lastname: Yousuf
authors.forename: Mohamed
authors.initials: M
authors.lastname: Shaker
authors.forename: Ahmed
authors.initials: A
authors.lastname: Eldorry
mesh_terms: D000328:Adult; D006502:Budd-Chiari Syndrome / Q000453:epidemiology* / Q000209:etiology / Q000235:genetics* / Q000473:pathology; D004534:Egypt / Q000453:epidemiology; D005165:Factor V / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D042965:Methylenetetrahydrofolate Reductase (NADPH2) / Q000235:genetics; D009154:Mutation; D011247:Pregnancy; D011248:Pregnancy Complications / Q000235:genetics; D012307:Risk Factors; D055815:Young Adult
chemical_list: C095381:factor V Leiden; D005165:Factor V; D042965:Methylenetetrahydrofolate Reductase (NADPH2)