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.