Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome

Sakr, M. S.; Reda, Mohga A.; Ebada, Hend E.; Abdelmoaty, Ahmed S.; Hefny, Zeinab M.; Ibrahim, Zakaria Hassan; Aboelmaaty, Mohamed E.

doi:10.1016/j.clinre.2019.10.006

Cited by 10 publications

(4 citation statements)

References 26 publications

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“…Prognosis is poor with hepatic failure, but there is also a 'silent' form with a relatively better outcome [2] . Although BD is a relatively uncommon (<5%) cause of BCS in Europe, it is more frequent (9-14%) as a cause of BCS in endemic nations like Turkey [4,5] . Patients with BCS should be evaluated for BD, especially young adult male patients if they present indicative symptoms including IVC thrombosis and recurring oral or genital ulcers, as in our case [4] .…”

Section: Discussionmentioning

confidence: 99%

Behçet's Disease-related Budd–Chiari Syndrome Successfully Managed with anti-TNF Antibody: A Case Report and Review of the Literature

Erul

Uzun

Köksal

et al. 2022

European Journal of Case Reports in Internal Medicine

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Behçet's disease (BD) is multisystemic vasculitis with heterogeneous clinical manifestations. We describe the case of a 26-year-old man who presented with Budd–Chiari syndrome (BCS) related to BD. The patient received infliximab (IFX) due to the severity of vascular involvement. Subsequently, after IFX therapy, hospital-acquired pneumonia, trapped lung, and fungal infection of the lung and central nervous system developed as complications. The patient benefited from a second course of IFX and clinical remission was achieved following early identification and treatment of complications. Data on the presentation and prognosis of BCS related to BD are extremely limited. Our case report supports the growing evidence that anti-TNF antibody is a promising treatment for BD-related BCS.

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Section: Discussionmentioning

confidence: 99%

Behçet's Disease-related Budd–Chiari Syndrome Successfully Managed with anti-TNF Antibody: A Case Report and Review of the Literature

Erul

Uzun

Köksal

et al. 2022

European Journal of Case Reports in Internal Medicine

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“…Compared with PVT, there is a greater prevalence of association with myeloproliferative neoplasm (30%-57% of cases)[ 17 , 94 ]. Some acquired thrombophilic conditions, such as paroxysmal nocturnal hemoglobinuria and Behçet’s disease have also a higher causative link in BCS compared with PVT (12% vs < 1%, respectively)[ 95 - 97 ]. To the contrary, BCS caused by local factors is rare, with the only exception of hepatic hydatid cysts in countries where Echinococcus granulosus is endemic[ 98 ].…”

Section: Bcsmentioning

confidence: 99%

Direct oral anticoagulants for the treatment of splanchnic vein thrombosis: A state of art

Monaco,

Bucherini,

Stefanini

et al. 2023

World J Gastroenterol

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Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious diagnoses such as Hirschsprung’s disease (HD), meconium ileus due to Cystic Fibrosis, etc. The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis, such as anorectal manometry or rectal suction biopsy. What if there was another etiology of perinatal constipation, that is far lesser known? Cow’s milk protein allergy (CMPA) is often diagnosed in infants within the first few weeks of life, however, there are studies that show that the CMPA allergen can be passed from mother to an infant in-utero, therefore allowing symptoms to show as early as day one of life. The presentation is more atypical, with perinatal constipation rather than with bloody stools, diarrhea, and vomiting. The diagnosis and management would be avoidance of cow's milk protein within the diet, with results and symptom improvement in patients immediately. Therefore, we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in patients. This entails first ruling out CMPA with safe, noninvasive techniques with diet modification, and if unsuccessful, then moving forward with further diagnostic modalities.

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“…A small (<3 cm) tumor size was chosen to match the size of benign hepatocellular lesions in BCS (10). Malignancy was confirmed by histologic evaluation, iodized oil accumulation after transcatheter arterial embolization therapy, increased serum AFP level that decreased immediately after transcatheter arterial embolization; criteria for HCC diagnosis on MRI included: TlWI hypo-, iso-, or hyperintensity to the liver, T2WI hyperintensity to the liver, and contrast-enhanced Tl arterial enhancement with washout on portal venous and delayed phase (11).…”

Section: Study Populationmentioning

confidence: 99%

Differentiation between small hepatocellular carcinoma (<3 cm) and benign hepatocellular lesions in patients with Budd-Chiari syndrome: the role of multiparametric MR imaging

Zandieh,

Shahbazian,

Tang

et al. 2023

Front. Oncol.

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ObjectiveTo investigate the value of multiparametric MR imaging to differentiate between small hepatocellular carcinoma (s-HCC) versus benign liver lesions in patients with Budd-Chiari syndrome.Methods12 patients with benign hepatocellular lesions and 32 patients with small (<3 cm) HCCs were assessed. MRI images were reviewed by two radiologists blinded to the patient background information; lesion T1 and T2 signal intensities and ADC values were compared with the background liver. Enhancement of lesion relative to hepatic parenchyma [(T1Enh-T1liver)/T1liver] in the arterial, venous, and delayed phases was also compared between the two groups. A multivariable logistic model was developed using these categorical measures; the predictive value of the model was tested using the Area Under the Receiver operating characteristic (AU-ROC) curve for logistic models. P-values <0.05 were considered statistically significant.ResultsThere were consistent differences in T1lesion/T1liver, and T2lesion/T2liver, and ADClesion/ADCliver between benign hepatocellular lesions versus the sHCC group (p<0.001, p<0.001, p = 0.045, respectively). Lesion-to-background liver enhancement in the portal venous and delayed phases was different between the benign lesions versus sHCC (p=0.001). ROC analysis for the logistic model that included the T1 ratio, T2 ratio, and portal venous enhancement ratio demonstrated excellent discriminatory power with the area under the curve of 0.94.ConclusionMultiparametric MR imaging is a useful method to help differentiate benign liver lesions from sHCC in patients with Budd-Chiari syndrome.

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Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome

Cited by 10 publications

References 26 publications

Behçet's Disease-related Budd–Chiari Syndrome Successfully Managed with anti-TNF Antibody: A Case Report and Review of the Literature

Behçet's Disease-related Budd–Chiari Syndrome Successfully Managed with anti-TNF Antibody: A Case Report and Review of the Literature

Direct oral anticoagulants for the treatment of splanchnic vein thrombosis: A state of art

Differentiation between small hepatocellular carcinoma (<3 cm) and benign hepatocellular lesions in patients with Budd-Chiari syndrome: the role of multiparametric MR imaging

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