Epidemiological aspects of Budd-Chiari in Egyptian patients: A single-center study

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%

NA: Not available; PC: Protein C; PS: Protein S; AT: Antithrombin; FVLM: Factor V Leiden mutation; PGM: Prothrombin gene mutation; MTHFR: Methylene tetrahydrofolate reductase; JAK2: Janus tyrosine kinase-2; MPD: Myeloproliferative disorder; APA: Antiphospholipid antibody syndrome; PNH: Paroxysmal nocturnal hemoglobinuria.

¹Reported by Valla in 2009[1] according to Primignani et al[12,14], Patel et al[15], Colaizzo et al[16], and Kiladjian et al[17].