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2016 ; 8
(16
): 691-702
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Redefining Budd-Chiari syndrome: A systematic review
#MMPMID27326316
Shin N
; Kim YH
; Xu H
; Shi HB
; Zhang QQ
; Colon Pons JP
; Kim D
; Xu Y
; Wu FY
; Han S
; Lee BB
; Li LS
World J Hepatol
2016[Jun]; 8
(16
): 691-702
PMID27326316
show ga
AIM: To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari
syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same
disorder. METHODS: A systematic review of observational studies conducted in
adult subjects with primary BCS, hepatic vein outflow tract obstruction,
membranous obstruction of the inferior vena cava (IVC), obliterative
hepatocavopathy, or HVT during the period of January 2000 until February 2015 was
conducted using the following databases: Cochrane Library, CINAHL, MEDLINE,
PubMed and Scopus. RESULTS: Of 1299 articles identified, 26 were included in this
study. Classical BCS is more common in women with a pure hepatic vein obstruction
(49%-74%). HVC-BCS is more common in men with the obstruction often located in
both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents
with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with
chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms
(MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F
mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F
mutation in 2%-5%. Classical BCS responds well to medical management alone and
1(st) line management of HVC-BCS involves percutaneous recanalization, with few
managed with medical management alone. CONCLUSION: Systematic review of recent
data suggests that classical BCS and HVC-BCS may be two clinically different
disorders that involve the disruption of hepatic venous outflow.