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. 2011 Nov 14;17(42):4704-10.
doi: 10.3748/wjg.v17.i42.4704.

Epidemiological aspects of Budd-Chiari in Egyptian patients: a single-center study

Mohammad Sakr  1 , Eman Barakat, Sara Abdelhakam, Hany Dabbous, Said Yousuf, Mohamed Shaker, Ahmed Eldorry
Affiliations

Epidemiological aspects of Budd-Chiari in Egyptian patients: a single-center study

Mohammad Sakr et al. World J Gastroenterol. .

Abstract

Aim: 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).

Conclusion: 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.

Keywords: Budd-Chiari syndrome; Epidemiological aspects; Etiology; Factor V Leiden mutation; Methylene tetrahydrofolate reductase gene mutation.

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Figures

Figure 1
Figure 1
Representative color Doppler ultrasonograph showing a dilated congested left hepatic vein with significant stenosis at the junction thereof with the inferior vena cava.
Figure 2
Figure 2
Representative B-mode sonograph showing occlusion of all hepatic veins, a slit-like inferior vena cava and a markedly enlarged caudate lobe.
See this image and copyright information in PMC

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