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2016 ; 16
(10
): e32457
Nephropedia Template TP
gab.com Text
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English Wikipedia
The Long-Term Outcomes for Patients with Budd-Chiari Syndrome Caused by Behcet s
Disease: A Case Series on the Results, from Cirrhosis to Death
#MMPMID27882061
Cansu DU
; Temel T
; Erturk A
; Kasifoglu T
; Acu B
; Korkmaz C
Hepat Mon
2016[Oct]; 16
(10
): e32457
PMID27882061
show ga
BACKGROUND: Budd-Chiari syndrome, which is a rare complication of Behcet's
disease, carries a high mortality rate. OBJECTIVES: The aim of the study was to
present our long-term follow up experience with patients suffering from
Budd-Chiari syndrome due to Behcet's disease. METHODS: The records of 402
patients with Behcet's disease were evaluated retrospectively. To facilitate
detection of the long-term complications caused by Budd-Chiari syndrome, the
patients were evaluated via physical examinations, laboratory tests, imaging
modalities, and endoscopy results. RESULTS: The data for 402 patients diagnosed
with Behcet's disease, who were followed up at our hospital over 16 years, were
analyzed retrospectively. Five of these 402 patients (1.2%) were diagnosed as
having Budd-Chiari syndrome. The patients with Budd-Chiari syndrome were aged
between 23 and 54, and all five were male. The interval between the onset of
Behcet's disease and the development of Budd-Chiari syndrome ranged from 1 to 8
years. All the patients had combined venous occlusion (affecting the hepatic vein
and inferior vena cava). Portal venous thrombosis was detected in only one
patient (Case 1), who died 1 month after the diagnosis of Budd-Chiari syndrome.
The survival time for the other four patients after the diagnosis of Budd-Chiari
syndrome ranged from 4 to 16 years. During the long-term follow-up, hepatic
masses were detected via radiological surveillance in Case 3 (in the form of
large regenerative nodules) and Case 4 (nodular regenerative hyperplasia and
cirrhosis). CONCLUSIONS: In our study, portal venous thrombosis was detected in
the patient who died during the acute period only. A study including large
numbers of Budd-Chiari-syndrome patients with Behcet's disease and portal venous
thrombosis would be helpful to determine the prognostic significance of portal
venous thrombosis in Budd-Chiari-syndrome patients with Behcet's disease. In
addition, patients should be monitored regularly for the development of hepatic
masses via a long-term surveillance program.