Epidemiological Characteristics and Etiology of Budd-Chiari Syndrome in Upper Egypt

Figures & data

Table 1 Baseline Sociodemographic and Laboratory Features of the Included Patients (n=50)

M/F (n)	28/22
Distribution by age, n(%)
<20 yr	5 (10%)
20–29	15 (30%)
30–40	19 (38%)
> 40	11 (22%)
Laboratory parameters	Mean & Std. Deviation
WBCs (×10^3/L)	5.7 ± 1.7
Hb(g/dl)	10.7 ± 1.9
Platelets(×10^3/L)	164 ± 53
Bilirubin (mg/dl) Total	1.07 ± 1.24
Direct bilirubin (mg/dl)	0.57 ± 0.89
ALT (U/L)	41.47± 5.37
AST (U/L)	42.2±96.5
Albumin (g/dl)	3.1 ± 2.4
INR	1.38 ± 0.31
Creatinine (mg/dl)	1.23 ± 0.98
MELD score	12.5 ± 5.5
Child Class (n= 50)
Non cirrhotic n,(%)	9 (18%)
Child class B n,(%)	8(16%)
Child class C n,(%)	33(66%)

Table 2 Etiology of Budd-ChiariSyndrome in the Studied Patients

Etiology	n(%)
Isolated protein C deficiency	13(26)
No identified etiology	11(22)
Isolated protein S deficiency	5(10)
Membranous web	4(8)
Factor V Leiden mutation (Heterozygous)	5(10)
Antiphospholipid Syndrome	4(8)
Hormonal therapy(females)	3(6)
Behςet’s Disease	2(4)
Combined protein C and S deficiency	1(2)
Combined protein C and S deficiency and antithrombin III	1(2)
Combined protein C and antithrombin III deficiency	1(2)

Table 3 Relation Between Etiology and Gender

Etiology	Males n(%)	Females n(%)	χ^2	P value	Sig
Isolated protein C deficiency	11(84.62)	2(15.38)	12.00	0.001	VHS
No identified etiology	6(54.55)	5(45.45)	10.00	0.002	VHS
Isolated protein S deficiency	0(0)	5(100)	5.00	0.025	S
Membranous web	4(100)	0(0)	4.00	0.046	S
Factor V Leiden mutation (Heterozygous)	4(80)	1(20)	4.00	0.046	S
Antiphospholipid syndrome	0(0)	4(100)	4.00	0.046	S
Hormonal therapy(females)	0(0)	3(100)	3.00	0.083	NS
Behςet’s disease	1 (50)	1(50)	0.00	1.000	NS
Combined protein C and S deficiency	1(100)	0(0)	1.00	0.317	NS
Combined protein C and S and antithrombin III deficiency	1(100)	0(0)	1.00	0.317	NS
Combined protein C and antithrombin III deficiency	0(0)	1(100)	1.00	0.317	NS

Abbreviations: NS, not significant; S, significant; VHS, very highly significant.

Table 4 Clinical Presentations of Budd-Chiari Syndrome in the Studied Patients (N= 50)

Symptoms	n(%)
Abdominal pain	48(96)
Abdominal distension	41(82)
Hematemesis and/or melena	18(36)
History of previous thrombosis	5(10)
History of recurrent abortion	3(6)
Recurrent oral and genital ulcer (Behςet’s disease)	2(4)
Signs
Ascites	41(82)
Lower limb oedema	34(68)
Hepatomegaly	25(50)
Splenomegaly	21(42)
Dilated abdominal veins	20(40)
Hepatic encephalopathy	18(36)
Jaundice	8(16)
Leg ulcers	1(2)

Figure 1 CT findings in BCS: (A) Axial CT image in a patient with acute BCS shows thrombosis of main portal vein seen as filling defect (arrow, A). Coronal CT image in another patient with acute BCS: shows filling defect in the middle HV (arrow, B). Ascites is also seen. Axial and coronal CT images in a patient with thrombosis of IVC seen as filling defect causing expansion of the lumen (arrows, C and D).

Abbreviations: BCS, Budd-Chiari syndrome; HV, hepatic veins; IVC, inferior vena cava.

Figure 2 Trans-jugular (A) and trans-femoral (B) catheter venography of the IVC showing total occlusion of the suprahepatic portion of the IVC, with tight stenosis of its junction with the hepatic veins and multiple dilated collaterals.

Table 5 Distribution of the Site of Venous Obstruction as Detected by Radiological Assessment According to the Etiology of Budd-Chiari Syndrome

Etiology	HV Only n(%)	HV and IVC n(%)	IVC Only n(%)	χ^2	P value	Sig
Isolated Protein C deficiency	13(100)	0(0)	0(0)	14.00	0.001	VHS
Isolated protein S deficiency	4(80)	0(0)	1(20)	5.00	0.082	NS
Factor V Leiden mutation	5(100)	0(0)	0(0)	6.00	0.050	S
Antiphospholipid syndrome	2(50)	2(50)	0(0)	4.00	0.135	NS
Behcets disease	0(0)	2(100)	0(0)	3.00	0.083	NS
Non-identified etiology	11(100)	0(0)	0(0)	12.00	0.002	VHS
Hormonal therapy	2(66.67)	1(33.33)	0	3.00	0.223	NS
Membranous web	0(0)	0(0)	4(100)	5.00	0.082	NS
Combined protein C and S deficiency	1(100)	0(0)	0(0)	2.00	0.368	NS
Combined protein C and S and antithrombin III deficiency	1(100)	0(0)	0(0)	2.00	0.368	NS
Combined protein C and antithrombin III deficiency	1(100)	0(0)	0(0)	2.00	0.368	NS
Total (n%)	40(80)	5(10)	5(10)	49.00	0.000	VHS

Abbreviations: NS, not significant; S, significant; VHS, very highly significant.