- Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients. [Journal Article]
- CIClin Immunol 2018 Aug 17
- CONCLUSIONS: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.
- A report on three patients with Echinococcus multilocularis: Lessons learned. [Journal Article]
- IJIndian J Gastroenterol 2018 Aug 18
- Echinococcus multilocularis (EM) is the most virulent species of the genus Echinococcus. It causes a highly lethal helminthic disease in humans. The disease may present as hepatic mass mimicking a ma...
Echinococcus multilocularis (EM) is the most virulent species of the genus Echinococcus. It causes a highly lethal helminthic disease in humans. The disease may present as hepatic mass mimicking a malignant neoplasm. Due to the vascular and neural invasion, protean clinical manifestations including Budd-Chiari syndrome (BCS) may be the clinical presentation of this condition. We herein report three cases of Echinococcus multilocularis; the first case presenting as multiple hepatic space-occupying lesions, second as liver mass infiltrating the nerve bundles, and the third as a hepatic mass infiltrating the large vessels including inferior vena cava and right hepatic vein presenting as BCS. EM is a parasite with capabilities of mass-forming effect, neural and vascular invasion. Though cases of BCS have been described, most of these are due to secondary compression and rarely by direct parasitic invasion.
- Clinical Efficacy of Spectral Computed Tomography for Evaluating Liver Function in Patients with Budd-Chiari Syndrome. [Journal Article]
- ARAcad Radiol 2018 Aug 09
- CONCLUSIONS: NIC determined through spectral CT was beneficial for evaluating liver function in patients with BCS, and can provide imaging data for reexamination, prognostic evaluation, and follow-up of patients with BCS after treatment. Liver parenchyma NIC values in BCS patients with varying liver function may provide a degree of diagnostic value. Multi-locus and multi-parameter studies of spectral CT can help to further evaluate liver function and assess prognosis in patients with BCS.
- Surgical Techniques and Long-term Outcomes of Living-donor Liver Transplantation With Inferior Vena Cava Replacement Using Atriocaval Synthetic Interposition Graft for Budd-Chiari Syndrome. [Journal Article]
- AnnSAnn Surg 2018 Aug 03
- CONCLUSIONS: Our refined technique does not require unnecessary and dangerous dissection of the diseased IVC, and eliminates the residual suprahepatic vena cava with the possibility of BCS recurrence by connecting the graft to the healthy atrium.
- Ultrasonography in Liver Vascular Disease. [Journal Article]
- UMUltraschall Med 2018; 39(4):382-405
- Vascular liver diseases include a heterogeneous group of disorders affecting the micro- and the macro-circulation of the liver. Thrombosis and obstruction of the inflow (portal vein) and/or outflow v...
Vascular liver diseases include a heterogeneous group of disorders affecting the micro- and the macro-circulation of the liver. Thrombosis and obstruction of the inflow (portal vein) and/or outflow venous system (Budd-Chiari syndrome), spontaneous porto-systemic shunts, diseases affecting the sinusoids, and hepatic vascular malformations are the most important vascular liver diseases. Thrombosis of the portal venous system and of the hepatic venous system occur most commonly and are potentially life-threatening conditions, while congenital and acquired pro-thrombotic diseases are major causal factors, together with local factors triggering thrombotic events. Despite their overall low prevalence, vascular liver diseases represent the second cause of portal hypertension in the Western world. Imaging techniques are of paramount importance in the diagnostic process, as well as in the follow-up of patients affected by these conditions. In this review, we focus on the role of ultrasonography in the management of vascular liver diseases by highlighting advantages and drawbacks of this imaging technique. In addition, we provide a state of the art presentation of the possibilities offered by ultrasound in the evaluation of vascular and parenchymal features in vascular liver diseases encompassing not only the use of grayscale imaging, but also the application of Doppler ultrasound, the measurement of hemodynamic parameters and the assessment of liver stiffness.
- Transjugular Intrahepatic Portosystemic Shunt (TIPS): Pathophysiologic Basics, Actual Indications and Results with Review of the Literature. [Review]
- ROFORofo 2018; 190(8):701-711
- CONCLUSIONS: TIPS placement is an established procedure with a new indication as "early TIPS". In the European recommendations it is only the second-line therapy for prevention of recurrent variceal hemorrhage and for secondary prophylaxis in patients with refractory ascites although several studies showed a clear benefit of the TIPS procedure compared to ligation and NSBBs.
- Long-term Outcomes of Endoluminal Sharp Recanalization of Occluded Inferior Vena Cava in Budd-Chiari Syndrome. [Journal Article]
- JLJ Laparoendosc Adv Surg Tech A 2018 Jul 23
- CONCLUSIONS: Endoluminal sharp recanalization of occluded IVC in patients with BCS is safe and effective with good long-term outcomes.
- Surgical Treatment of Pulmonary Complications in Behçet's Syndrome. [Journal Article]
- STSemin Thorac Cardiovasc Surg 2018 Jul 19
- We described disease characteristics, management and outcome of a group of Behçet's syndrome (BS) patients who underwent pulmonary lobectomy, segmentectomy or various pleura interventions for complic...
We described disease characteristics, management and outcome of a group of Behçet's syndrome (BS) patients who underwent pulmonary lobectomy, segmentectomy or various pleura interventions for complications due to pulmonary artery or descending aorta involvement. There were 9 patients with BS (8 M/1 F; mean age: 24.8 ± 7.5 and mean disease duration: 4.3 ± 3.8 years) who underwent lung surgery from 2000 to 2017. Their medical files including operation details and radiological and pathological studies were reviewed retrospectively. Lobectomy was done in 6 patients, decortications and pleura interventions in the remaining 3. The reason for the surgical procedures were giant pulmonary arterial aneurysms refractory to the medical treatment (n = 4), pneumothorax due to large cavities (n = 2), pleural effusions refractory to the medical treatment (n = 1), a bronchopleural fistula after embolization (n = 1) and bronchiectasis (n = 1). All patients received medical treatment with cyclophosphamide and corticosteroids before the surgical interventions. The histological examination in patients with pseudo-aneurysms showed destruction of medial elastic fibers, inflammatory cell infiltration of adventitial tissues and penetration into the adjacent bronchi with adherent thrombi. Two patients died: one due to Budd-Chiari syndrome 1 year after the surgery, another because of a massive hemoptysis 3 months after lobectomy. The remaining 7 patients are still alive with a median follow-up of 8 years (inter-quartile range [IQR]:4-11). Lobectomies in BS patients with giant pulmonary aneurysms refractory to medical treatment can be done with favorable outcome. Also, complications such as large cavities causing pneumothorax, refractory pleural effusions, bronchiectasis, and embolization complications can be managed effectively with surgical interventions.
- Hepatic vein stenting in a 7 week/old infant with Budd-Chiari syndrome using an anterograde approach from the inferior accessory hepatic vein. [Journal Article]
- DLDig Liver Dis 2018 Jun 18
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- Transplant-free Survival in Chronic Liver Disease Presenting as Acute Liver Failure in Childhood. [Journal Article]
- TTransplantation 2018 Jul 19
- CONCLUSIONS: In children ALF-CLD is common, has peculiar features and is associated with a favourable outcome. This study suggests the need to distinguish this entity from other forms of ALF in children.