9,585 results match your criteria Behcet Disease


Budd-Chiari syndrome in Behcet's disease: A report of two cases.

Exp Ther Med 2019 Mar 24;17(3):1737-1741. Epub 2018 Dec 24.

Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China.

Budd-Chiari syndrome (BCS) is a rare but severe venous form of Behcet's disease (BD) that is caused by the obstruction of the venous outflow tract that transports blood from hepatic veins into the inferior vena cava. In countries where BD is prevalent, including the Middle East and Far East, BCS awareness is important. In the present study, two cases of BCS are presented in two male Chinese patients with BD. Read More

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http://dx.doi.org/10.3892/etm.2018.7130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364229PMC

Increased vein wall thickness in Behçet disease.

J Vasc Surg Venous Lymphat Disord 2019 Feb 15. Epub 2019 Feb 15.

Academic Hospital, Istanbul, Turkey.

Objective: Lower extremity (LE) deep venous thrombosis (DVT) is the main feature of vascular involvement in Behçet disease (BD). We thought that vein wall thickness (VWT) could be a surrogate marker for venous inflammation and hence predict future vascular involvement. We assessed VWT in proximal LE veins in BD patients without DVT, BD patients with DVT, and healthy controls in a formal, masked protocol. Read More

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http://dx.doi.org/10.1016/j.jvsv.2018.11.006DOI Listing
February 2019

Open surgical repair of abdominal aortic aneurysms in Behçet's disease.

J Med Vasc 2019 Feb 19;44(1):9-18. Epub 2018 Dec 19.

Vascular surgery department, faculty of medicine and pharmacy of Fez, Sidi Mohamed Ben Abdellah University, UHC Hassan II, Fez, Morocco.

Background: Behçet's disease (BD) is a multisystem chronic autoimmune inflammatory disorder that involves multiple organs. Arterial involvement in BD is rare. Aortic involvement is one of the most severe manifestations and is associated with a much higher mortality. Read More

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http://dx.doi.org/10.1016/j.jdmv.2018.11.007DOI Listing
February 2019
1 Read

[Diagnostic Strategy and Therapeutic Dilemma in Acute Posterior and Panuveitis].

Klin Monbl Augenheilkd 2019 Feb 14. Epub 2019 Feb 14.

Augenklinik, Universitätsspital Basel, Schweiz.

Acute posterior and panuveitis mostly affect younger patients and affect both eyes in more than half of cases. Because of the severe consequences in the clinical course, rapid and broad differential diagnosis are critical steps. Permanent loss of vision after a delay in starting therapy and the initiation of ineffective treatment are both serious risks. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0828-7395
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http://dx.doi.org/10.1055/a-0828-7395DOI Listing
February 2019
1 Read

Filter-associated inferior vena cava thrombosis with duodenal perforation: case report and literature review.

Ann Vasc Surg 2019 Feb 11. Epub 2019 Feb 11.

Department of Thoracic and Vascular Surgery, Arnaud de Villeneuve Hospital, Montpellier, France.

Objectif: The aim of this paper is to report a case of filter associated inferior vena cava (IVC) thrombosis with perforation of the duodenum and penetration of a vertebral body by the filter struts.

Case Report: A 37 year old woman with a medical history of Behcet`s disease treated with corticosteroids, underwent placement of a retrievable IVC filter because of recurrent ileofemoral venous thrombosis regardless of therapeutic levels of anticoagulation. Despite a correct positioning of the filter, the second follow-up CT-scan, performed at 1 year, showed a complete thrombosis of the infra-renal IVC segment, with perforation of the vessel wall by the filter struts and penetration in the duodenum. Read More

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http://dx.doi.org/10.1016/j.avsg.2018.11.021DOI Listing
February 2019
2 Reads

Phenotypes in Behçet's syndrome.

Authors:
Emire Seyahi

Intern Emerg Med 2019 Feb 11. Epub 2019 Feb 11.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, 81310, Istanbul, Turkey.

Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. Read More

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http://dx.doi.org/10.1007/s11739-019-02046-yDOI Listing
February 2019
2 Reads

Genotyping of Italian patients with Behçet syndrome identified two novel ERAP1 polymorphisms using sequencing-based approach.

Hum Immunol 2019 Feb 8. Epub 2019 Feb 8.

Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital of Potenza, via Potito Petrone, Potenza, 85100, Italy; Basilicata Ricerca Biomedica (BRB) Foundation, Matera, 75100, Italy. Electronic address:

The endoplasmic reticulum aminopeptidase protein 1 gene (ERAP1) is related to several human diseases, including Behçet syndrome (BS), a multisystemic disorder with unknown etiology. ERAP1 is involved in immune response and its role can be influenced by gene single nucleotide variations (SNVs). We genotyped the ERAP1 whole structure in 50 consecutive BS patients and 50 ethnically-matched healthy controls using both bioinformatics and molecular methodologies. Read More

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http://dx.doi.org/10.1016/j.humimm.2019.02.003DOI Listing
February 2019
1 Read

Pathergy testing: prospective comparison of dermatoscopic evaluation and naked eye examination.

Intern Emerg Med 2019 Feb 8. Epub 2019 Feb 8.

Department of Dermatology, Medicalpark Bahcelievler Private Hospital, Istanbul, Turkey.

Pathergy phenomenon is a non-specific tissue hyperreactivity reaction due to trauma and is a minor diagnostic criterion of Behcet's disease. In this study, 100 patients with a suspicion of Behcet's disease who were referred to Cerrahpasa Medical Faculty Dermatology department between 01.11. Read More

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http://dx.doi.org/10.1007/s11739-019-02039-xDOI Listing
February 2019
2 Reads

The role of vitamin D receptor gene polymorphisms in the pathogenesis of Behçet's disease: A case-control study in Turkish population.

Ann Hum Genet 2019 Feb 7. Epub 2019 Feb 7.

Department of Clinical Immunology, School of Medicine, Ankara University, Ankara, Turkey.

Background: Behçet's disease (BD) is an inflammatory disorder characterized by oral aphthous lesions, uveitis, and genital ulcerations. The vitamin D receptor (VDR) has a crucial role in the pathogenesis of this disease because it mediates the functions of vitamin D in the immune system. Alterations of VDR expression related to polymorphic alleles of the VDR gene may play a pathogenic role in BD and BD's clinical presentations. Read More

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http://dx.doi.org/10.1111/ahg.12301DOI Listing
February 2019
2 Reads

The colonization rate of needleless connector and the impact of disinfection for 15 s on colonization: A prospective pre- and post-intervention study.

J Vasc Access 2019 Feb 5:1129729819826036. Epub 2019 Feb 5.

3 Department of Pediatric Intensive Care Unit, Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, Izmir, Turkey.

Purpose:: The optimal scrubbing time for the disinfection of the surface of needleless connectors has not been determined. The evidence for successful needleless connector decontamination with 70% isopropyl alcohol ranges from 5 to 60 s. The aim of this prospective study was to identify colonization on the external surface of needleless connectors on central venous catheters and measure the efficiency of 15 s of scrubbing with 70% alcohol. Read More

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http://dx.doi.org/10.1177/1129729819826036DOI Listing
February 2019
2 Reads

Auto-immunity profile evaluation during different clinical manifestations of Behçet disease in Algerian patients: effect of corticosteroid treatment.

Inflammopharmacology 2019 Feb 5. Epub 2019 Feb 5.

Cytokines and NO-Synthases, Laboratory of Cellular and Molecular Biology (LBCM), Faculty of Biological Science, USTHB, PB 32, 16111, Algiers, Algeria.

Background And Aims: Behçet disease (BD) is a chronic multisystem disease. It stands at the crossroads between the auto-immunity and auto-inflammatory disorders. Our study aims to evaluate corticosteroids therapy effects on serum immunoglobulin isotypes and anti-phospholipid auto-anti-body production in Algerian BD patients with different clinical manifestations. Read More

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http://dx.doi.org/10.1007/s10787-019-00567-8DOI Listing
February 2019
1 Read

Optical coherence tomography angiography findings in Behcet patients.

Int Ophthalmol 2019 Feb 1. Epub 2019 Feb 1.

Department of Ophthalmology, Ege University School of Medicine, İzmir, Turkey.

Purpose: Retinal vasculitis and vein occlusions are common causes of serious visual loss in Behçet's disease. We aimed to evaluate the optical coherence tomography angiography (OCTA) findings of Behcet uveitis (BU) patients.

Methods: We evaluated 32 eyes of 16 patients with BU and 30 eyes of 15 healthy controls. Read More

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http://dx.doi.org/10.1007/s10792-019-01080-1DOI Listing
February 2019
1 Read

Stroke revealing Neuro-Behçet's disease with parenchymal and extensive vascular involvement.

J Neurol Sci 2019 Jan 26;398:131-134. Epub 2019 Jan 26.

Neurology's Department, Mongi Ben Hmida National Institute of Neurology, Tunis, Tunisia.

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http://dx.doi.org/10.1016/j.jns.2019.01.044DOI Listing
January 2019

Reliability and accuracy of smartphones for paediatric infectious disease consultations for children with rash in the paediatric emergency department.

BMC Pediatr 2019 Jan 31;19(1):40. Epub 2019 Jan 31.

Department of Pediatric Emergency Department, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Objective: Smartphones and associated messaging applications have become the most common means of communication among health care workers and the general population. The aim of this study was to evaluate the reliability and accuracy of smartphones for the diagnosis of rash in children admitted to emergency departments during the night shift.

Methods: The images of the children who were admitted to the paediatric emergency department with rash were included in this study, and at least two images taken with smartphones by residents or paediatric infectious disease fellows were re-directed to the chief consultant of the Paediatric-Infectious Department via smartphone. Read More

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http://dx.doi.org/10.1186/s12887-019-1416-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354364PMC
January 2019
2 Reads

Comparative analysis of anxiety-depressive spectrum disorders in patients with rheumatic diseases.

Ter Arkh 2018 May;90(5):30-37

Federal State Budgetary Scientific Institution «Research Institute of Rheumatology named after V.A. Nasonova», Moscow, Russia.

Aim: Research objective - comparative analysis of incidence and structure of anxiety-depressive spectrum disorders (ADD) in patients with various rheumatic diseases (RD).

Materials And Methods: 613 patients with RD were enrolled in the study: 180 with a reliable diagnosis of systemic lupus erythematosus (SLE), 128 with rheumatoid arthritis (RA), 110 with systemic sclerosis (SSc), 115 with Behcet's disease (BD), 80 with primary Sjögren's syndrome (pSS). Female prevailed in all groups (95% of patients with pSS, 88,2% - SSc, 87,2% - RA, 85,5% of SLE) except BD patients (70% male). Read More

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http://dx.doi.org/10.26442/terarkh201890530-37DOI Listing
May 2018
1 Read

The association of inflammatory markers and echocardiographic parameters in Behçet's disease.

Acta Cardiol 2019 Jan 28:1-8. Epub 2019 Jan 28.

a Department of Cardiology , Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital , Istanbul , Turkey.

Background: The main objective of the current study is to find out if any association exists between specific inflammatory markers such as homocysteine (Hcy) and pentraxin-3 (PTX-3) and cardiac involvement determined by means of echocardiographic parameters in patients with Behçet disease (BD).

Methods: From January 2011 to January 2012, a total of 62 Behçet's patients were enrolled in the study. Thirty-two healthy subjects constituted the control group. Read More

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http://dx.doi.org/10.1080/00015385.2018.1560071DOI Listing
January 2019
1 Read
0.561 Impact Factor

A 3-Year Retrospective Study of the Epidemiology of Acute Respiratory Viral Infections in Pediatric Patients With Cancer Undergoing Chemotherapy.

J Pediatr Hematol Oncol 2019 Jan 25. Epub 2019 Jan 25.

Pediatric Infection.

Background: Acute viral respiratory infections are common causes of febrile episodes in children. There are still limited data about distribution of acute viral respiratory infections in children with cancer.

Objective: The first aim of this study was to evaluate the viral etiology and seasonality of acute viral respiratory infection in pediatric patients with cancer in a 3-year study. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001418DOI Listing
January 2019
5 Reads

Quality of Life and Depression in Turkish Patients with β-Thalassemia Major: A Cross-Sectional Study.

Hemoglobin 2018 Sep - Nov;42(5-6):326-329. Epub 2019 Jan 23.

b Clinic of Pediatric Hematology-Oncology, Sağhk Bakanlığı Üniveristesi, İzmir Dr. Behçet Uz Çocuk Sağlığı ve Cerrahisi Eğitim Araştırma Hastanesi , İzmir , Turkey.

Thalassemias are the most common monogenic disorders worldwide. Thalassemia patients experience difficulties in their schooling, finding jobs and/or marriage because of functional and physical limitations caused by this disease. It is expected that the quality of life (QoL) of patients with thalassemia will be lower than those without this disease. Read More

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http://dx.doi.org/10.1080/03630269.2018.1551231DOI Listing
January 2019
4 Reads
0.955 Impact Factor

Cardiac and vascular complications of Behçet disease in the Tunisian context: clinical characteristics and predictive factors.

Adv Rheumatol 2018 Oct 1;58(1):32. Epub 2018 Oct 1.

Internal Medicine and Endocrinology Department, Fattouma Bourguiba University Hospital, 1st June Avenue, 5000, Monastir, Tunisia.

Background: Cardiac and vascular involvement in Behçet disease (BD), also referred as vasculo BD, is frequent. We aimed to describe clinical characteristics, predictive factors and management of vasculo BD in the Tunisian context.

Methods: We retrospectively studied 213 records of all BD patients followed between January 2004 and May 2016 in the Internal Medicine Department and who fulfilled the ISGBD criteria. Read More

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http://dx.doi.org/10.1186/s42358-018-0032-xDOI Listing
October 2018
1 Read

Association between Human Leukocyte Antigens with Autoimmune Diseases: Diagnostic Approach.

Curr Rheumatol Rev 2019 Jan 15. Epub 2019 Jan 15.

Thalassemia and Hemoglobinopathy Research Center, Research Institute of Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz. Iran.

Background: The process of antigen presentation to immune cells is an undeniable contributor to pathogenesis of autoimmune diseases. Different studies have indicated several factors that are related to autoimmunity. Human leukocyte antigens (HLAs) are among such factors, which have a key role in autoimmunity because of their involvement in antigen presentation process. Read More

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http://dx.doi.org/10.2174/1573397115666190115143226DOI Listing
January 2019
5 Reads

Clinical Significance of Serum Bilirubin in Behçet's Disease.

Authors:
Tuba Tülay Koca

J Transl Int Med 2018 Dec 31;6(4):185-188. Epub 2018 Dec 31.

Department of Physical Medicine and Rehabilitation, Sütçü İmam University,Kahramanmaras, Turkey.

Background And Objective: Bilirubin (Bb) is the product of the intravascular compartment of catabolic pathway. In a small number of clinical trials, it has been shown that Bb molecules are associated with cardiovascular diseases, diabetes, cancer, autoimmune (lupus, rheumatoid arthritis) diseases and schizophrenia. Behçet's disease is a chronic, multisystemic, inflammatory vasculitis that was first described by Hulusi Behçet in 1937, which affects almost all organs and systems without any known aetiology. Read More

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http://dx.doi.org/10.2478/jtim-2018-0034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326032PMC
December 2018
7 Reads

[Vasculitis mimics].

Z Rheumatol 2019 Feb;78(1):24-30

Internistisch-rheumatologische Praxis, Jürgensallee 44, 22609, Hamburg, Deutschland.

Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse. Read More

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http://link.springer.com/10.1007/s00393-018-0581-8
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http://dx.doi.org/10.1007/s00393-018-0581-8DOI Listing
February 2019
7 Reads

Central Nervous System Peripheral T Cell Lymphoma Manifesting as Lymphomatosis Cerebri That Was Misdiagnosed as Neuro-Behçet's Disease: A Case Report.

Case Rep Oncol 2018 Sep-Dec;11(3):806-813. Epub 2018 Nov 29.

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan.

Background: Lymphomatosis cerebri (LC) is a unique form of primary central nerves lymphoma (PCNSL), which presents as diffuse infiltration of lymphoma cells characteristically in the white matter rather than tumor formation. However, the involvement of central nervous system (CNS) is unclear because of the lack of contrast enhancement.

Case Presentation: We treated a 53-year-old woman with LC and brain lesions resembling neuro-Behcet's disease. Read More

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http://dx.doi.org/10.1159/000495033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323408PMC
November 2018
6 Reads

Clinical and Genetic Aspects of Behçet's Disease in Japan.

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Stem Cell and Immune Regulation, Yokohama City University, Graduate School of Medicine, Japan.

Patients with Behçet's disease (BD) suffer from episodic ocular and mucocutaneous attacks, resulting in a reduced quality of life. The phenotype of Japanese BD has been changing over the past 20 years, and the rate of HLA-B*51-positive complete type is decreasing while that of intestinal type is increasing. This phenotypical evolution may be related to changes in as-yet-unknown environmental factors, as the immigration influx in Japan is low. Read More

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http://dx.doi.org/10.2169/internalmedicine.2035-18DOI Listing
January 2019
4 Reads

Serum calprotectin and ischemia modified albumin levels as markers of disease activity in Behçet's disease.

Postepy Dermatol Alergol 2018 Dec 13;35(6):609-613. Epub 2018 Nov 13.

Department of Rheumatology, Ankara Numune Training and Research Hospital, Ankara, Turkey.

Introduction: Behçet's disease (BD) is a complex multisystemic inflammatory disorder which is characterized by recurrent attacks of acute inflammation. As there is no universally recognized pathognomonic laboratory marker of BD, its diagnosis is still based on clinical findings.

Aim: To evaluate the role of calprotectin and ischemia modified albumin (IMA) as biomarkers in the assessment of disease activity of BD. Read More

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http://dx.doi.org/10.5114/pdia.2017.71269DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320477PMC
December 2018
6 Reads

[The etiology, diagnosis, and treatment of neurological complications in Behçet disease and its related disorder Sweet disease].

Authors:
Kinya Hisanaga

Rinsho Shinkeigaku 2019 Jan 29;59(1):1-12. Epub 2018 Dec 29.

Departments of Neurology and Clinical Research Center, National Hospital Organization Miyagi Hospital.

Behçet disease, and its related disorder Sweet disease, are multisystem inflammatory conditions characterized by muco-cutaneous symptoms. When neuropsychiatric symptoms appear, the two conditions are referred to as neuro-Behçet disease and neuro-Sweet disease. While diagnosing these conditions according to their diagnostic criteria, muco-cutaneous symptoms must be observed; however, neuropsychiatric symptoms may precede muco-cutaneous symptoms. Read More

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http://dx.doi.org/10.5692/clinicalneurol.cn-001238DOI Listing
January 2019
1 Read

Effect of Interferon alfa-2a Treatment on Adaptive and Innate Immune Systems in Patients With Behçet Disease Uveitis.

Invest Ophthalmol Vis Sci 2019 Jan;60(1):52-63

Department of Ophthalmology, School of Medicine, Koc University, Istanbul, Turkey.

Purpose: To investigate the effect of interferon alfa-2a on T regulatory (Treg) cells, T helper 17 (Th17) cells, and expression of Toll-like receptors (TLRs) in Behçet disease (BD) patients with uveitis.

Methods: Twenty-seven patients who received interferon alfa-2a for active BD uveitis despite conventional immunomodulatory therapies and healthy controls were enrolled. Peripheral blood Treg and Th17 cell frequencies were determined by flow cytometry as gated cells for CD3+CD4+Foxp3+ and CD3+CD4+IL17A+, respectively. Read More

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http://dx.doi.org/10.1167/iovs.18-25548DOI Listing
January 2019
1 Read
3.404 Impact Factor

Use of Apremilast for Aphthous Ulcers in a Patient With Behçet’s Syndrome

J Drugs Dermatol 2018 Dec;17(12):1328-1329

Behçet’s syndrome is an inflammatory disease characterized by aphthous oral ulcers and several systemic manifestations, which include genital ulcers, ocular disease, skin lesions, arthritis, and vascular disease Read More

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December 2018
5 Reads

Echocardiographic features of interventricular septal dissection in patients with Behçet's disease.

Echocardiography 2019 02 25;36(2):394-400. Epub 2018 Dec 25.

Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Involvement of the heart in Behçet's disease (BD) is rare. We retrospectively analyzed these three patients with interventricular septal (IVS) dissection in BD and discussed the echocardiographic manifestations of IVS dissections. In our patients, the echocardiographic characteristics of IVS dissection were echo-free space in the IVS basal segment or basal to middle segment, dilatation in the diastole and contraction in systole, and abnormal turbulent blood flow in the heart. Read More

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http://dx.doi.org/10.1111/echo.14235DOI Listing
February 2019
2 Reads

Intra- and interreader reproducibility of PI-RADSv2: A multireader study.

J Magn Reson Imaging 2018 Dec 21. Epub 2018 Dec 21.

Molecular Imaging Program, National Cancer Institute, NIH, Bethesda, Maryland, USA.

Background: The Prostate Imaging Reporting and Data System version 2 (PI-RADSv2) has been in use since 2015; while interreader reproducibility has been studied, there has been a paucity of studies investigating the intrareader reproducibility of PI-RADSv2.

Purpose: To evaluate both intra- and interreader reproducibility of PI-RADSv2 in the assessment of intraprostatic lesions using multiparametric magnetic resonance imaging (mpMRI).

Study Type: Retrospective. Read More

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http://doi.wiley.com/10.1002/jmri.26555
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http://dx.doi.org/10.1002/jmri.26555DOI Listing
December 2018
10 Reads

Surgical treatment of intestinal perforation in Behçet Syndrome: an unusual presentation.

Pan Afr Med J 2018 26;30:230. Epub 2018 Jul 26.

Hospital de Cascais, Cascais, Portugal.

Behçet syndrome is a chronic, recurring, systemic disorder characterized by the histopathologic finding of nonspecific vasculitis in multiple organs. Behçet syndrome involves the gastrointestinal tract in 10-50% of patients; The main sites of involvement are the terminal ileum and cecum. In patients with Behçet syndrome, CT is advocated for early detection of complications as well as for exclusion of other abdominal pathologic conditions but there is no specific exam. Read More

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http://dx.doi.org/10.11604/pamj.2018.30.230.15950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295296PMC
January 2019
2 Reads

Clinical characteristics of avascular necrosis in patients with Behçet disease: a case series and literature review.

Rheumatol Int 2019 Jan 17;39(1):153-159. Epub 2018 Dec 17.

Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, 06100, Ankara, Turkey.

Avascular necrosis (AVN), also known as osteonecrosis, is characterized by death of the osteocytes due to inadequate blood supply caused by various mechanisms. The hip is the most common affected joint followed by knee. Incidence of AVN in rheumatic diseases is variable and high corticosteroid (CS) therapy is a known major risk factor for development of AVN. Read More

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http://link.springer.com/10.1007/s00296-018-4224-9
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http://dx.doi.org/10.1007/s00296-018-4224-9DOI Listing
January 2019
5 Reads

Vasculitis in Systemic Autoinflammatory Diseases.

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

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http://dx.doi.org/10.3389/fped.2018.00377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287042PMC
December 2018
15 Reads

Olfactory bulb volume and olfactory sulcus depth in patients with Behçet's disease.

J Laryngol Otol 2018 Dec 18:1-5. Epub 2018 Dec 18.

ENT Clinics,Adana Numune Training and Research Hospital,Adana,Turkey.

Objective: To investigate olfactory bulb volume and olfactory sulcus depth in patients with Behçet's disease, using magnetic resonance imaging.

Methods: Cranial magnetic resonance imaging scans of 27 adults with Behçet's disease (10 males and 17 females) and 27 healthy controls were examined. Olfactory bulb volume and olfactory sulcus depth were measured on coronal, T2-weighted, spectral pre-saturation with inversion recovery sequences. Read More

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http://dx.doi.org/10.1017/S0022215118002141DOI Listing
December 2018
1 Read

Sudden unilateral vision loss in an adult with Behcet disease: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13594

Department of Ophthalmology, Sichuan University West China Hospital, Sichuan, China.

Rationale: Behcet disease is an autoimmune vasculitis, involving multiple systems and organs. It is often chronic and recurrent, and involves all levels of arteries and veins.

Patient Concerns: A 40-year-old Chinese male with sudden vision loss in his right eye for 3 days was reported. Read More

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http://dx.doi.org/10.1097/MD.0000000000013594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320199PMC
December 2018
1 Read

[Reconstruction of scarring pharyngeal stenosis with forearm free flap:a case report].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Sep;32(18):1434-1435

A 33-year-old man presented with recurrent oral ulcers for more than 10 years,accompanying dyspnea and dysphagia for 1 year and aggravate for 1 month.Physical examination:pharyngeal cavity stenosis,mucous retention, epiglottis was extruded into the infant type. Electronic laryngoscope:Epiglottis curl,bilateral pyriform sinus disappeared. Read More

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http://dx.doi.org/10.13201/j.issn.1001-1781.2018.18.018DOI Listing
September 2018
1 Read

Increased IL-1β levels are associated with an imbalance of "oxidant/antioxidant" status during Behçet's disease.

Eur Cytokine Netw 2018 Sep;29(3):95-102

USTHB, "Cytokines and NO Synthases" team, LBCM, FSB, BP 32, El Alia, 16111, Bab ezzouar, Algiers, Algeria.

Behçet's disease is a multisystem disease. It stands at the crossroad between the autoimmunity and auto-inflammatory disorders. In this study, we sought to address a relationship that might exist between interleukin-1β (IL-1β) and the oxidants/antioxidants markers in Behçet's patients. Read More

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http://dx.doi.org/10.1684/ecn.2018.0411DOI Listing
September 2018
2 Reads

Adalimumab efficacy for venous thrombosis in typical sites in Behçet's syndrome.

Arthritis Rheumatol 2018 Dec 7. Epub 2018 Dec 7.

Department of Neurosciences, Psychology, Pharmacology and Child Health (NEUROFARBA), University of Firenze, Italy.

In our work entitled "Adalimumab-based treatment versus DMARDs for venous thrombosis in Behçet syndrome. A retrospective study of 70 patients with vascular involvement" , we showed that Adalimumab (ADA)-based regimen is more effective and rapid than disease modifying anti-rheumatic drugs (DMARDs) in inducing resolution of venous thrombosis in patients with Behçet syndrome (BS), also allowing significant reduction of steroid exposure. This article is protected by copyright. Read More

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http://dx.doi.org/10.1002/art.40794DOI Listing
December 2018
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Update on the epidemiology, risk factors and disease outcomes of Behçet's disease.

Authors:
Nurullah Akkoç

Best Pract Res Clin Rheumatol 2018 04 21;32(2):261-270. Epub 2018 Sep 21.

Izmir, Turkey. Electronic address:

Behçet's disease (BD) may be regarded as a polygenic autoinflammatory disease although adaptive immune system has also been implicated in pathogenesis. Different classification criteria sets exist for BD, including the new "International Criteria for BD." The pooled prevalence of BD was calculated as 10. Read More

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http://dx.doi.org/10.1016/j.berh.2018.08.010DOI Listing
April 2018
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Chronic granulamatous disease: Two decades of experience from a paediatric immunology unit in a country with high rate of consangineous marriages.

Scand J Immunol 2019 Feb 23;89(2):e12737. Epub 2019 Jan 23.

Department of Pediatric Immunology, Ege University Faculty of Medicine, Izmir, Turkey.

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by susceptibility to bacterial and fungal infections resulting from the inadequacy of phagocytic leucocytes to produce reactive oxygen radicals. CGD is a genetically heterogeneous disease with an X-linked recessive (XR-CGD) form caused by mutations in the CYBB (OMIM #300481) gene encoding the gp91(phox) protein, and an autosomal recessive (AR-CGD) form caused by mutations in the CYBA (OMIM #608508), NCF1 (OMIM #608512), NCF2 (OMIM #608515) and NCF4 (OMIM #601488) genes encoding p22(phox), p47(phox), p67(phox) and p40(phox), respectively. The genetic mutation of one of the cytosolic p47phox/p67phox proteins and membrane-bound gp91phox/p22phox proteins, which constitutes the NADPH oxidase enzyme complex, causes the disease. Read More

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http://dx.doi.org/10.1111/sji.12737DOI Listing
February 2019
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Does illness perception associate with disease symptoms in Behçet's disease?

Intern Emerg Med 2018 Nov 30. Epub 2018 Nov 30.

Department of Dermatology, School of Medicine, Marmara University, Istanbul, Turkey.

This study aims to assess the relationship between illness perception and disease course and symptoms in Behçet disease (BD). One hundred ten consecutive BD patients (F/M 50/60, mean age 38.5 ± 9. Read More

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http://dx.doi.org/10.1007/s11739-018-1983-yDOI Listing
November 2018
3 Reads

Impact of central line bundle for prevention of umbilical catheter-related bloodstream infections in a neonatal intensive care unit: A pre-post intervention study.

Am J Infect Control 2018 Nov 27. Epub 2018 Nov 27.

Division of Pediatric Infectious Disease, Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, Izmir, Turkey.

Background: Central line-associated bloodstream infection is the most common infection associated with health care in preterm infants. The purpose of this 18-month cross-sectional study was to evaluate the effectiveness of bundle applications in the prevention of umbilical venous catheter (UVC)-associated bloodstream infections.

Methods: This study included patients in whom UVCs were inserted and who were diagnosed with central line-associated bloodstream infection between July 1, 2016, and December 31, 2017, according to the Centers for Disease Control and Prevention criteria. Read More

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http://dx.doi.org/10.1016/j.ajic.2018.10.002DOI Listing
November 2018
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GPR56 homozygous nonsense mutation p.R271* associated with phenotypic variability in bilateral frontoparietal polymicrogyria.

Turk J Pediatr 2018 ;60(3):229-237

Departments of Medical Biology and Genetics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.

Öncü-Öner T, Ünalp A, Porsuk-Doru İ, Ağılkaya S, Güleryüz H, Saraç A, Ergüner B, Yüksel B, Hız-Kurul S, Cingöz S. GPR56 homozygous nonsense mutation p.R271* associated with phenotypic variability in bilateral frontoparietal polymicrogyria. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.001DOI Listing
January 2018
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LACC1 Regulates TNF and IL-17 in Mouse Models of Arthritis and Inflammation.

J Immunol 2019 Jan 3;202(1):183-193. Epub 2018 Dec 3.

Department of Human Genetics, Genentech, Inc., South San Francisco, CA 94080.

Both common and rare genetic variants of laccase domain-containing 1 (, previously C13orf31) are associated with inflammatory bowel disease, leprosy, Behcet disease, and systemic juvenile idiopathic arthritis. However, the functional relevance of these variants is unclear. In this study, we use LACC1-deficient mice to gain insight into the role of LACC1 in regulating inflammation. Read More

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http://dx.doi.org/10.4049/jimmunol.1800636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305794PMC
January 2019
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Vascular Behçet's syndrome: an update.

Intern Emerg Med 2018 Nov 29. Epub 2018 Nov 29.

Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.

Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the most frequent vascular involvements, affecting altogether 15-40% of BS patients. Atypical thrombosis is also an important clinical feature of BS, involving the vascular districts of the inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right ventricle. Read More

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http://dx.doi.org/10.1007/s11739-018-1991-yDOI Listing
November 2018
11 Reads

Behçet's disease and breast cancer: A case series study.

J Cancer Res Ther 2018 Oct-Dec;14(6):1184-1190

Department of Medical Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Introduction: The relation between Behçet's disease (BD) and breast cancer (BC) is unclear. Our purpose is to investigate whether BD has an important effect on BC or vice versa.

Patients And Methods: A total of 12 female BC patients with a diagnosis of BD were identified from a cohort including 5050 BC patients. Read More

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http://dx.doi.org/10.4103/0973-1482.189249DOI Listing
November 2018
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Coexistence of Gaucher Disease and severe congenital neutropenia.

Blood Cells Mol Dis 2018 Aug 10. Epub 2018 Aug 10.

Ege University Medical Faculty, Pediatric Metabolism Department, İzmir, Turkey.

Gaucher Disease (GD) is the most common lysosomal storage disorder has traditionally been classified into three clinical phenotypes. Type 3 GD is characterized by neurological involvement but neurological symptoms generally appear later in life than in type 2 disease. Neutropenia is much rarer than other hematological manifestations in GD and has not been scrutinized adequately. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10799796183005
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http://dx.doi.org/10.1016/j.bcmd.2018.07.001DOI Listing
August 2018
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New data in causes of autoinflammatory diseases.

Joint Bone Spine 2018 Nov 22. Epub 2018 Nov 22.

Centre national de référence des maladies auto-inflammatoires et de l'amylose inflammatoire (CEREMAIA), 94270 Le Kremlin-Bicêtre, France; Cellules souches, plasticité cellulaire, médecine régénératrice et immunothérapies, Inserm, Université de Montpellier, 34090 Montpellier, France; Département de génétique médicale, maladies rares et médecine personnalisée, CHU de Montpellier, 34295 Montpellier, France.

The spectrum of factors known to mediate autoinflammation has broadened recently to include not only interleukin-1 (IL-1) and interferon, but also abnormalities that impair NF-κB pathway negative regulation. The NF-κB pathway is activated upon contact of a ligand with tumor necrosis factor receptor 1 (TNFR1) and plays a pivotal role in triggering the inflammatory process by producing major cytokines such as IL-1, IL-6, and TNF. Negative regulation of the NF-κB pathway, which is essential to stop the inflammatory process, depends on the level of ubiquitination of the proteins associated with TNFR1 and of other intermediate compounds. Read More

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http://dx.doi.org/10.1016/j.jbspin.2018.11.003DOI Listing
November 2018
15 Reads

Efficacy of seeds oil in patients with Behcet's disease: a double-blind randomized controlled trial.

Avicenna J Phytomed 2018 Nov-Dec;8(6):498-503

Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.

Objective: (NS) is a herbal medicine with anti-inflammatory and anti-oxidant functions. This study was designed to evaluate the effect of oral administration of NS seeds oil on the treatment of Behcet's disease (BD).

Materials And Methods: In this double-blind randomized controlled study, 130 patients with BD were screened and 71 patients with BD were randomly allocated to the treatment (n=37) and control (n=34) groups. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6235660PMC
November 2018
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Behçet's Disease - Case Presentation and Review Literature.

Open Access Maced J Med Sci 2018 Oct 3;6(10):1871-1874. Epub 2018 Oct 3.

University Clinical Center of Kosovo, Clinic of Dermatology, Prishtina, Kosovo.

Background: Behçet's syndrome is associated with inflammation of various areas of the body. Sy. Behcet is a rare, chronic, recurrent disease characterised by changes in the: Arteries that supply blood to the body's tissues, veins that take the blood back to the lungs, the back of the eye's retina, brain, joints, skin and bowels. Read More

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http://dx.doi.org/10.3889/oamjms.2018.393DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236042PMC
October 2018
8 Reads