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World J Gastroenterol. Nov 14, 2011; 17(42): 4704-4710
Published online Nov 14, 2011. doi: 10.3748/wjg.v17.i42.4704
Published online Nov 14, 2011. doi: 10.3748/wjg.v17.i42.4704
Table 1 Relevant clinical data on patients with Budd-Chiari syndrome (n = 94)
Symptom | n (%) |
Abdominal enlargement | 84 (89.4) |
Abdominal pain | 78 (83) |
History of previous thrombosis | 26 (27.7) |
Recurrent abortion (females; 58) | 14 (24.1) |
Gastrointestinal bleeding | 15 (15.9) |
Recurrent oral and/or genital ulcers | 13 (13.8) |
Signs | |
Ascites | 80 (85.1) |
Hepatomegaly | 78 (83) |
Splenomegaly | 48 (51.1) |
Lower limb edema | 46 (48.9) |
Dilated abdominal veins | 39 (41.5) |
Jaundice | 36 (38.3) |
Abdominal tenderness | 34 (36.2) |
Encephalopathy | 29 (30.9) |
Table 2 The primary etiologies of Budd-Chiari syndrome (n = 94)
Etiology | n (%) | |
FVLM (64 tested) | Homozygous | 10 (15.6) |
Heterozygous | 24 (37.5) | |
MTHFR (60 tested) | Homozygous | 8 (13.3) |
Heterozygous | 23 (38.3) | |
PGM (60 tested) | Homozygous | 1 (1.7) |
Heterozygous | 2 (3.3) | |
JAK2 (MPD) (62 tested) | + | 18 (29) |
Primary APA | + | 16 (17) |
Secondary APA | + | 11 (11.7) |
Behçet’s disease | + | 12 (12.8) |
Protein C deficiency | + | 4 (4.3) |
Antithrombin III deficiency | + | 4 (4.3) |
Protein S deficiency | + | 1 (1.1) |
PNH | + | 2 (2.1) |
Hormonal therapy (58 females) | + | 9 (15.5) |
Pregnancy-related (58 females) | + | 10 (17.2) |
Table 3 Relationship between gender and etiology in patients with Budd-Chiari syndrome (n = 94)
Etiology | Gender n (%) | χ² | P value | Sig | ||
Male | Female | |||||
PC deficiency | + | 1 (2.90) | 3 (5.20) | 1 | NS | |
PS deficiency | + | 1 (2.90) | 0 (0.00) | 0.38 | NS | |
AT III deficiency | + | 2 (5.70) | 2 (3.40) | 0.63 | NS | |
FVLM | Homozygous | 5 (20.80) | 5 (12.50) | 1.77 | 0.18 | NS |
Heterozygous | 6 (25.00) | 18 (45.00) | ||||
PGM | Homozygous | 0 (0.00) | 1 (2.60) | 1.54 | 0.21 | NS |
Heterozygous | 0 (0.00) | 2 (5.10) | ||||
MTHFR | Homozygous | 6 (28.60) | 2 (5.10) | 8.41 | 0.01 | HS |
Heterozygous | 9 (42.90) | 14 (35.90) | ||||
JAK2 (MPD) | + | 6 (28.60) | 12 (29.30) | 0.003 | 0.95 | NS |
Primary APA | + | 5 (13.90) | 11 (19.00) | 0.4 | 0.52 | NS |
Secondary APA | + | 1 (2.80) | 10 (17.20) | 0.05 S | ||
Behçet’s disease | + | 11 (30.60) | 1 (1.70) | < 0.001 VHS | ||
PNH | + | 1 (2.80) | 1 (1.70) | 1 NS |
Table 4 Duplex ultrasound findings in patients with Budd-Chiari syndrome (n = 94)
Ultrasound finding | Patients n (%) | |
Hepatomegaly | 78 (83) | |
Splenomegaly | 48 (51.1) | |
No. of instances of occluded HV | 0 | 3 (3.2) |
1 | 2 (2.1) | |
2 | 12 (12.8) | |
3 | 77 (81.9) | |
RHV occlusion | 85 (90.4) | |
MHV occlusion | 91 (96.8) | |
LHV occlusion | 87 (92.6) | |
IVC occlusion | 22 (23.4) | |
PV occlusion | 5 (5.3) | |
Anatomical localization of thrombosis at presentation | Isolated HV | 70 (74.5) |
Combined HV and IVC | 16 (17) | |
Isolated IVC | 3 (3.2) | |
Associated PV thrombosis | 5 (5.3) |
Table 5 Relationship between etiology and radiological findings [occluded vein(s)] in patients with Budd-Chiari syndrome
Etiology | Anatomical localization of thrombosis at presentation n (%) | ||||||||
HV only | HV and PV | HV, PV and IVC | HV and IVC | IVC | χ2 | P value | Sig | ||
PC deficiency | + | 3 (4.3) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 0 (0.0) | 0.001 | 0.99 | NS |
PS deficiency | + | 1 (1.4) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0.32 | 0.58 | NS |
AT III Deficiency | + | 3 (4.3) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 0 (0.0) | 0.001 | 0.99 | NS |
FVLM | Homo | 9 (17.0) | 0 (0.0) | 1 (12.5) | 0 (0.0) | 9 (17.0) | 0.03 | 0.85 | NS |
Hetero | 19 (35.8) | 2 (100.0) | 3 (37.5) | 0 (0.0) | 19 (35.8) | ||||
PGM | Homo | 1 (2.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (2.0) | 0.52 | 0.47 | NS |
Hetero | 2 (4.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2 (4.0) | ||||
MTHFR | Homo | 7 (14.0) | 0 (0.0) | 1 (14.3) | 0 (0.0) | 7 (14.0) | 0.29 | 0.59 | NS |
Hetero | 19 (38.0) | 0 (0.0) | 3 (42.9) | 1 (10.0) | 19 (38.0) | ||||
JAK2 (MPD) | + | 16 (31.4) | 2 (100.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2.08 | 0.15 | NS |
Primary APA | + | 9 (12.9) | 0 (0.0) | 1 (33.3) | 6 (37.5) | 0 (0.0) | 2.58 | 0.11 | NS |
Secondary APA | + | 9 (12.9) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 1 (33.3) | 0.02 | 0.88 | NS |
Behçet’s disease | + | 2 (2.9) | 0 (0.0) | 2 (66.7) | 7 (43.8) | 1 (33.3) | 21.25 | < 0.0001 | HS |
PNH | + | 2 (2.9) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0.62 | 0.43 | NS |
Table 6 Etiologies of Budd-Chiari syndrome described in previous studies and in the current work
Etiology | Reported rate1 | Rate in current study | |
PC deficiency | 20% | 4.30% | |
PS deficiency | 7% | 1.10% | |
AT III deficiency | 5% | 4.30% | |
FVLM | Homozygous | NA | 15.60% |
Heterozygous | 20% | 37.50% | |
PGM | Homozygous | NA | 1.70% |
Heterozygous | 7% | 3.30% | |
MTHFR | Homozygous | NA | 13.30% |
Heterozygous | NA | 38.30% | |
JAK2-positive (MPD) | 50% | 29% | |
Primary APA | 10% | 17% | |
Secondary APA | 11.70% | ||
Behçet’s disease | 5% | 12.80% | |
PNH | 2% | 2.10% | |
Hormonal therapy (58 females) | 50% | 15.50% | |
Non-identified etiology | 5% | 8.50% |
- Citation: Sakr M, Barakat E, Abdelhakam S, Dabbous H, Yousuf S, Shaker M, Eldorry A. Epidemiological aspects of Budd-Chiari in Egyptian patients: A single-center study. World J Gastroenterol 2011; 17(42): 4704-4710
- URL: https://www.wjgnet.com/1007-9327/full/v17/i42/4704.htm
- DOI: https://dx.doi.org/10.3748/wjg.v17.i42.4704