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2017 ; 96
(34
): e7425
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Liver cirrhosis caused by chronic Budd-Chiari syndrome
#MMPMID28834866
Lin M
; Zhang F
; Wang Y
; Zhang B
; Zhang W
; Zou X
; Zhang M
; Zhuge Y
Medicine (Baltimore)
2017[Aug]; 96
(34
): e7425
PMID28834866
show ga
Chronic Budd-Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and
tends to be misdiagnosed in clinical practice. In order to characterize LC caused
by chronic BCS, we conducted this retrospective observational study. Medical
records of all patients who were initially diagnosed as chronic BCS with LC when
discharged from our department from January, 2011 to October, 2016 were reviewed.
Cirrhotic patients with known causes and cases lacked key data were excluded.
Data of remaining patients was collected and analyzed. A total of 15 cases were
included in this study. Patients with LC caused by chronic BCS were characterized
by preserved liver function and prominent portal hypertension (PH). Abdominal
distention and edema of lower extremities were most common initial
manifestations. Intra- or extrahepatic collaterals on imaging studies were of
great importance for differential diagnosis. Most of these patients received
interventional angioplasty followed by anticoagulation with warfarin and survived
without obvious complications of PH. Chronic BCS was a rare but important cause
of LC and should always be considered in patients with chronic liver disease and
so-called cryptogenic LC. Early diagnosis and timely treatment may improve
outcome. Correct interpretation of imaging examinations was fundamental to
avoiding misdiagnosis.