Aetiological factors of Budd-Chiari syndrome in Algeria

Table 1 Revealing symptoms

Symptoms	n	%
Ascites	86	74.8
Abdominal pain	49	42.6
Jaundice	16	13.9
Bleeding	14	12.2
Hepatic encephalopathy	6	5.2
Fever	18	15.7
Lower limb edema	15	13

Table 2 Clinical and laboratory characteristics at diagnosis

Demographic parameters
Age (yr)	34 (16-72)
M/F (n)	45/70
Distribution by age, n (%)
< 20 yr	8 (9)
20-40 yr	63 (72)
> 40 yr	29 (34)
Clinical parameters n (%)
Disease onset
Acute	8.7 (10)
Chronic	63.5 (73)
Subacute	27.8 (32)
Ascites	62.6 (72)
Hepatomegaly	62.6 (72)
Splenomegaly	42.6 (49)
Thoraco-abdominal venous collaterals	43.5 (50)
Lumber venous collaterals	11.3 (13)
Hepatic encephalopathy	3.5 (4)
Jaundice	40 (46)
Lower limb oedema	24.3 (28)
Biological parameters
ALT (ULN)	2.5 (1-60)
Bilirubin (mg/dL)	20.58 (3-265)
Prothrombin time (%)	57 (14-98)
Haemoglobin (g/dL)	11.7 (3-17)
RBC (106/mm3)	4.5 (1.7-7.9)
WBC (103/mm3)	7.7 (1.3-21.5)
Platelet count (/mm3)	263212 (29000-695000)
Albumin (g/L)	29.4 (16.7-45)
Thrombocytosis n (%)	20 (23)
Erythrocytosis n (%)	11.3 (13)
Hyperleucocytosis n (%)	25.2 (29)
Cholestasis n (%)	73.9 (85)
Hyperbilirubinaemia n (%)	41.7 (48)
Elevated liver enzymes n (%)	49.6 (57)
Liver failure n (%)	61.7 (71)
Renal failure n (%)	9.5 (11)

ALT: Alanine aminotransferase; ULN: Upper limit of normal; M/F: Male/female; RBC: Red blood cells; WBC: White blood cells.

Table 3 Radiological features

Thrombosis site	n	%
Hepatic veins	83	72.2
IVC	3	2.6
IVC and HV	29	25.2
Associated thrombosis	37/113	32.2
Retrohepatic IVC	7	6.0
Portal vein	26	22.6
Mesenteric vein/splenic vein	4	3.4
Renal veins	4	3.4
Iliac veins	3	2.6

IVC: Inferior vena cava; HV: Hepatic veins.

Table 4 Budd-Chiari syndrome aetiologies

Aetiologies	Tested patients	n	% (n/tested n)
MPD	104	36	34.6
Patent		19
Latent		17
APL syndrome	92	20	21.7
Protein C deficiency	67	13	19.4
Protein S deficiency	59	5	8.5
Antithrombin deficiency	68	0	0
APCR	68	7	10.3
Celiac disease	88	10	11.4
Hyperhomocysteinaemia	42	5	11.9
PNH	11	4
Systemic disease1	106	6	5.6
Inflammatory bowel disease2	60	5	8.3
Gene II mutation	21	1	4.7
Liver hydatic cyst	115	5	4.3
Hepatocellular carcinoma	115	1	0.8
Hormonal factors	70	25	35.7
Oral contraception	70	24	34.3
Pregnancy	70	3	4.3
Hormonal treatment	70	1	1.4
Unknown aetiology		24	20.9

¹Systemic lupus erythematosus (n = 1), granulomatosis (n = 1), sarcoidosis (n = 1), Behçet disease (n = 3);

²Ulcerative colitis (n = 1), Crohn’s disease (n = 4). PNH: Paroxysmal nocturnal haemoglobinuria; APL: Anti-phospholipid syndrome; APCR: Activated protein C resistance; MPD: Myeloproliferative disease.