Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt

Table 1 Aetiology and clinical presentation of Budd-Chiari syndrome patients at baseline

	All patients	Subgroups
		Medical therapy only	TIPS
	(n = 21)	(n = 7)	(n = 14)
Male/female	6/15	3/4	3/11
Age (yr) at first contact, median (range)	40 (17-66)	41 (17-64)	38 (20-66)
Aetiology, n (%)
Thrombophilia1	7 (33)	2	5
Protein C deficiency	1 (5)	0	1
Protein S deficiency	1 (5)	1	0
Factor V leiden mutation	1 (5)	1	0
Hyperhomocysteinemia	2 (10)	0	2
Paroxysmal nocturnal haemoglobinuria	2 (10)	0	2
Myeloproliferative disorder	4 (19)	2	2
Polycythemia vera	4 (19)	2	2
Other	4 (19)	4	0
Diabetic ketoacidosis	3 (14)	3	0
Angio leiomyosarcoma in caval vena	1 (5)	1	0
Unknown2	7 (33)	0	7
More than one predisposing risk factors	1 (5)3	13	0
Clinical presentation, n (%)
Ascites	15 (71)	3	12
Abdominal pain	14 (67)	6	8
Hepatomegaly	11 (52)	4	7
Jaundice	1 (5)	0	1
Hepatic encephalopathy	1 (5)	0	1
Variceal bleeding	0	0	0

¹13 patients were screened for thrombophilic disorders;

²6 of 7 with unknown risk factors were screened for thrombophilic disorders with negative result;

³This patient suffered from polycythemia vera and had a Factor V Leiden mutation. TIPS: Transjugular intrahepatic portosystemic shunt.

Table 2 Diuretics treatment before and after transjugular intrahepatic portosystemic shunt treatment

	Before TIPS	After TIPS
Any diuretics treatment (n/n)	13/14	10/14
Furosemide dose [n/n,	12/14	3/14
median (range), mg/d]	80 (40-240)	200 (40-240)
Spironolactone dose [n/n,	12/14	9/14
median (range), mg/d]	175 (25-200)	100 (25-200)

TIPS: Transjugular intrahepatic portosystemic shunt.

Table 3 Biochemical changes before transjugular intrahepatic portosystemic shunt and during follow up

	Reference interval	Before TIPS	6 mo	12 mo	24 mo	36 mo
n		14	11	11	11	10
Alanine aminotransaminase (U/L)	(10-70) (M)/(10-45) (F)	73 (15-533)	41 (23-66)	34 (22-62)	42 (19-58)	35 (11-77)
Bilirubin (μmol/L)	5-25	35 (10-86)	28 (10-50)	27 (4-94)	25 (12-152)	21 (14-85)
Alkaline phosphatase (U/L)	35-105	180 (22-1105)	182 (86-391)	186 (94-461)	154 (88-358)	154 (89-349)
Albumin (μmol/L)	542-722	466 (331-711)	595 (439-707)	601 (554-714)	605 (532-684)	622 (497-727)
Creatinine (μmol/L)	(60-105) (M)/(45-90) (F)	76 (19-119)	71 (51-106)	69 (49-98)	71 (47-98)	59 (45-86)

Results are given as median (range). M: Male reference values, F: Female reference values; TIPS: Transjugular intrahepatic portosystemic shunt.