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Behcet syndrome

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https://www.readbyqxmd.com/read/27924945/a-low-balance-between-microparticles-expressing-tissue-factor-pathway-inhibitor-and-tissue-factor-is-associated-with-thrombosis-in-beh%C3%A3-et-s-syndrome
#1
E Khan, N L Ambrose, J Ahnström, A P Kiprianos, M R Stanford, D Eleftheriou, P A Brogan, J C Mason, M Johns, M A Laffan, D O Haskard
Thrombosis is common in Behçet's Syndrome (BS), and there is a need for better biomarkers for risk assessment. As microparticles expressing Tissue Factor (TF) can contribute to thrombosis in preclinical models, we investigated whether plasma microparticles expressing Tissue Factor (TF) are increased in BS. We compared blood plasma from 72 healthy controls with that from 88 BS patients (21 with a history of thrombosis (Th+) and 67 without (Th-). Using flow cytometry, we found that the total plasma MP numbers were increased in BS compared to HC, as were MPs expressing TF and Tissue Factor Pathway Inhibitor (TFPI) (all p < 0...
December 7, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27886800/beh%C3%A3-et-s-disease-how-to-diagnose-and-treat-vascular-involvement
#2
REVIEW
Emire Seyahi
Behçet's disease is a multisystem disorder with unknown etiology and a unique geographic distribution. The disease is characterized by recurrent skin-mucosa lesions and sight-threatening panuveitis. Vascular involvement, which is more common and more severe among males, has also equally characteristic features such as affecting mostly veins, having a significant tendency for thrombosis, and running a relapsing course. Lower extremity vein thrombosis (LEVT) is the most frequent manifestation, followed by vena cava inferior thrombosis...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27882061/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
#3
Dondu Uskudar Cansu, Tuncer Temel, Adem Erturk, Timucin Kasifoglu, Berat Acu, Cengiz Korkmaz
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...
October 2016: Hepatitis Monthly
https://www.readbyqxmd.com/read/27858841/marshall-syndrome-in-a-young-child-a-reality-case-report
#4
Laura Mihaela Trandafir, Madalina Ionela Chiriac, Smaranda Diaconescu, Ileana Ioniuc, Ingrith Miron, Daniel Rusu
BACKGROUND: Recurrent fever syndrome, known as the Marshall syndrome (MS), is a clinical entity that includes several clinical features, such as: fever (39-40°C) that occurs repeatedly at variable intervals (3-8 weeks) and in episodes of 3 to 6 days, cervical adenopathy, pharyngitis, and aphthous stomatitis. The diagnosis of MS is one of exclusions; laboratory data is nonspecific and no abnormalities correlated with MS have been detected thus far. METHODS: The authors report the case of a 2-year-old girl admitted to a tertiary pediatric center for repeated episodes of fever with aphthous stomatitis and laterocervical adenopathy...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27848185/pediatric-intestinal-beh%C3%A3-et-disease-complicated-by-myeloid-malignancies
#5
Kiichiro Kanamitsu, Akira Shimada, Ritsuo Nishiuchi, Tomonari Shigemura, Yozo Nakazawa, Kenichi Koike, Yuichi Kodama, Yuichi Shinkoda, Yoshifumi Kawano, Kozo Yasui, Koji Sasaki, Ryosuke Kajiwara, Hirokazu Tsukahara, Atsushi Manabe
Behçet disease (BD) is rarely seen in children. Its clinical manifestations are believed to differ between pediatric and adult patients. The characteristics of BD complicated by myelodysplastic syndrome (MDS) are well established for adult patients; however, because only a few cases of pediatric-onset BD complicated by MDS have been reported, its clinical characteristics remain unknown. We here retrospectively review pediatric-onset BD complicated by myeloid malignancies in Japan, having identified five such patients...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#6
Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, Birol Baysal
Behçet's disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet's disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27799881/accuracy-of-three-different-fecal-calprotectin-tests-in-the-diagnosis-of-inflammatory-bowel-disease
#7
Hui Won Jang, Hyun Sook Kim, Soo Jung Park, Sung Pil Hong, Tae Il Kim, Won Ho Kim, Jae Hee Cheon
BACKGROUND/AIMS: Several studies have found that the measurement of fecal calprotectin is useful for the early diagnosis of inflammatory bowel disease (IBD). We compared the effectiveness of three different fecal calprotectin kits for initial diagnosis in patients with suspected IBD. METHODS: We enrolled 31 patients with IBD (18 Crohn's disease [CD], 11 ulcerative colitis [UC], and two intestinal Behçet's disease), five with irritable bowel syndrome (IBS), and five with other colitis (four infectious colitis and one intestinal tuberculosis)...
October 2016: Intestinal Research
https://www.readbyqxmd.com/read/27791959/highlights-of-the-17th-international-conference-on-beh%C3%A3-et-s-syndrome
#8
REVIEW
Sinem Nihal Esatoglu, Gulen Hatemi, Pietro Leccese, Ignazio Olivieri
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September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27791958/one-year-in-review-2016-beh%C3%A3-et-s-syndrome
#9
REVIEW
Gulen Hatemi, Emire Seyahi, Izzet Fresko, Rosaria Talarico, Vedat Hamuryudan
Several articles highlighting the epidemiology, pathogenesis, clinical features, treatment modalities and disease assessment of Behçet's syndrome (BS) have been published during the last year. Clinical and radiological features of lower extremity deep vein thrombosis due to BS can be quite different than those found in thrombosis due to other causes; additionally, frequency of post-thrombotic syndrome is significantly increased in BS. Some clinical and colonoscopic features are useful in differentiating BS from Crohn's disease...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27791957/beh%C3%A3-et-s-syndrome-in-the-2000s-where-is-the-wisdom-we-have-lost-in-knowledge
#10
EDITORIAL
Hasan Yazici
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September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27791952/bronchial-artery-enlargement-may-be-the-cause-of-recurrent-haemoptysis-in-beh%C3%A3-et-s-syndrome-patients-with-pulmonary-artery-involvement-during-follow-up
#11
Sinem Nihal Esatoglu, Emire Seyahi, Serdal Ugurlu, Fatih Gulsen, Canan Akman, Murat Cantasdemir, Furuzan Numan, Hasan Tuzun, Melike Melikoglu, Hasan Yazici, Vedat Hamuryudan
OBJECTIVES: Haemoptysis occurring in a Behçet's syndrome (BS) patient with pulmonary artery involvement (PAI) during follow-up is usually regarded as PAI relapse. However, bronchial artery enlargement (BAE) may be the source of haemoptysis in some patients. METHODS: A chart review at the end of December 2014 revealed 118 patients with PAI in our centre since 1979. Nine (all men) had recurrent haemoptysis during follow-up which could not be explained with relapse of PAI...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27791948/the-association-between-the-parenchymal-neurological-involvement-and-posterior-uveitis-in-beh%C3%A3-et-s-syndrome
#12
Berivan Bitik, Abdurrahman Tufan, Kubilay Sahin, Yesim Sucullu Karadag, Sevinc Can Sandikci, Ridvan Mercan, Fikri Ak, Yasar Karaaslan, Mehmet Akif Ozturk, Berna Goker, Seminur Haznedaroglu
OBJECTIVES: Behçet's syndrome (BS) is a systemic vasculitis, which may involve multiple organ systems simultaneously. Clinical findings in BS often fit into well-recognized patterns, such as the association between papulo-pustular skin lesions and arthritis. We have recently observed a distinct pattern, in which a subtype of neuro-Behçet's syndrome (NBS) is often preceded by specific ophthalmic manifestations of the disease process. The purpose of this study is to evaluate the association between the parenchymal subtype of NBS and posterior uveitis (PU)...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27782772/clinical-patterns-and-causes-of-intraocular-inflammation-in-a-uveitis-patient-cohort-from-egypt
#13
Rowayda M Amin, Mohamed Gowieda, Ahmed Bedda, Ahmed Kamel, Alaa Radwan
PURPOSE: To analyze the patterns and causes of intraocular inflammation in patients attending uveitis referral clinics in Egypt. METHODS: The study included 454 patients with uveitis examined both at the Department of Ophthalmology, Alexandria Faculty of Medicine, and tertiary uveitis referral clinics in Cairo and the International Eye Clinic in Upper Egypt, between August 2013 and March 2016. All patients had a comprehensive ocular examination and systemic work-up...
October 26, 2016: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/27777341/identification-of-novel-biomarkers-for-behcet-disease-diagnosis-using-huprot-array-approach
#14
Chao-Jun Hu, Jian-Bo Pan, Guang Song, Xiao-Ting Wen, Zi-Yan Wu, Si Chen, Wen-Xiu Mo, Feng-Chun Zhang, Jiang Qian, Heng Zhu, Yong-Zhe Li
Behcet disease (BD) is a chronic systemic vasculitis and considered as an autoimmune disease. Although rare, BD can be fatal due to ruptured vascular aneurysms or severe neurological complications. To date, no known biomarker has been reported for this disease, making it difficult to diagnosis in the clinics. To undertake this challenge, we employed the HuProt arrays, each comprised of~20,000 unique human proteins, to identify BD-specific autoantibodies using a Two-Phase strategy established previously. In Phase I, we profiled the autoimmunity on the HuProt arrays with 75 serum samples collected from 40 BD patients, 15 diagnosed autoimmune patients who suffer from Takayasu arteritis (TA; N=5)), ANCA associated vasculitis(AAV; N=5), and Sjogren's syndrome(SS; N=5), and 20 healthy subjects, and identified 20 candidate autoantigens that were significantly associated with BD...
October 24, 2016: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/27755121/vasculitis-in-the-autoinflammatory-diseases
#15
Hagit Peleg, Eldad Ben-Chetrit
PURPOSE OF REVIEW: This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. RECENT FINDINGS: Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of the innate immune system. Despite the common occurrence of rashes and other skin lesions in these diseases, vasculitis is reported in only a few. On the other hand, neutrophilic dermatoses are more prevalent...
January 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27733942/the-role-of-mefv-mutations-in-the-concurrent-disorders-observed-in-patients-with-familial-mediterranean-fever
#16
Sabri Güncan, N Şule Y Bilge, Döndü Üsküdar Cansu, Timuçin Kaşifoğlu, Cengiz Korkmaz
OBJECTIVE: This study aimed to investigate the frequency in which familial Mediterranean fever (FMF) coexists with other diseases and determine whether Mediterranean fever (MEFV) gene mutations are involved in such coexistence. MATERIAL AND METHODS: In total, 142 consecutive patients with FMF investigated for MEFV mutation were enrolled in this study [Female: 87; Male: 55, mean age 32±12 years (11-62)]. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively...
September 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27699578/re-initiation-of-biologics-after-the-development-of-tuberculosis-under-anti-tnf-therapy
#17
Yesim Ozguler, Gulen Hatemi, Serdal Ugurlu, Emire Seyahi, Melike Melikoglu, Sermin Borekci, Ersan Atahan, Gul Ongen, Vedat Hamuryudan
The use of anti-TNF agents is associated with an increased risk of tuberculosis (TB) and anti-TNF agents are stopped when active TB develops. However, discontinuation of treatment can result in flare of the underlying disease. The charts of 22 patients who developed active TB among a cohort of 2754 patients using anti-TNF agents between 2001 and 2013 were reviewed retrospectively. Patients restarting biologics during further follow-up were identified. One patient with miliary TB died within 1 month. A biologic agent was restarted in 16 of the remaining 21 patients (76 %)...
December 2016: Rheumatology International
https://www.readbyqxmd.com/read/27684358/behcet-s-syndrome-in-nonendemic-regions
#18
Pietro Leccese, Yusuf Yazici, Ignazio Olivieri
PURPOSE OF REVIEW: Behcet's syndrome is more common in certain geographic regions, however, can be seen outside of these areas and need to be included in the differential diagnosis of many patients, as it has overlapping features with many rheumatologic conditions. RECENT FINDINGS: Especially in regions with immigrant populations, there seem to be similarities to originating countries in Behcet's prevalence, but the syndrome is not limited to those from certain backgrounds and can be seen in others also...
January 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27684357/systemic-vasculitis-and-the-gut
#19
Ibrahim Hatemi, Gulen Hatemi, Aykut F Çelik
PURPOSE OF REVIEW: Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. RECENT FINDINGS: Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield...
January 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27660198/pd-1-pd-l-and-autoimmunity-a-growing-relationship
#20
Mohammad Reza Zamani, Saeed Aslani, Arash Salmaninejad, Mohammad Reza Javan, Nima Rezaei
Programmed death 1 (PD-1) and its ligands, namely PD-L1 and PD-L2, are one of the key factors responsible for inhibitory T cell signaling, mediating the mechanisms of tolerance and providing immune homeostasis. Mounting evidence demonstrates that impaired PD-1:PD-L function plays an important role in a variety of autoimmune diseases such as Type 1 diabetes (T1D), encephalomyelitis, inflammatory bowel diseases (IBD), Rheumatoid Arthritis (RA), autoimmune hepatitis (AIH), Behcet's disease (BD), myasthenia gravis (MG), autoimmune uveitis (AU), Sjögren's syndrome (SjS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), myocarditis, and ankylosing spondylitis (AS)...
September 15, 2016: Cellular Immunology
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