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Behcet syndrome

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https://www.readbyqxmd.com/read/28283819/neurological-complications-of-beh%C3%A3-et-s-syndrome
#1
D P Kidd
In this review of the neurological complications of Behçet's syndrome, the clinical features and epidemiology of the systemic disease are summarised before a discussion of the neurological syndromes which may develop is made. Neurological involvement occurs in 9% of cases, and is equally prevalent in each geographical area. Vascular complications occur in 14%, in whom thrombosis of the venous sinuses or cerebral veins occurs, and intracranial hypertension, venous infarction and parenchymal haemorrhage may develop...
March 10, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28255922/the-clinical-and-pathological-characteristics-of-nephropathies-in-connective-tissue-diseases-in-the-japan-renal-biopsy-registry-j-rbr
#2
Kazunobu Ichikawa, Tsuneo Konta, Hiroshi Sato, Yoshihiko Ueda, Hitoshi Yokoyama
BACKGROUND: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE). METHODS: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). In total, 20,523 registered patients underwent biopsy between 2007 and 2013; from 110 patients with connective tissue diseases except SLE, we extracted data regarding the clinico-pathological characteristics of the renal biopsy...
March 2, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28250463/vasculitis-syndromes-shared-genetic-risk-for-beh%C3%A3-et-disease-and-crohn-s-disease
#3
Jessica McHugh
No abstract text is available yet for this article.
March 2, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28198796/carbonic-anhydrase-i-and-ii-autoantibodies-in-beh%C3%A3-et-s-disease
#4
Ahmet Mentese, Ahmet Alver, Selim Demir, Aysegul Sumer, Serap Ozer Yaman, Murat Karkucak, Arzu Aydin Capkin, Diler Us Altay, Ibrahim Turan
BACKGROUND: Behçet's disease is a vasculitis, seen more frequently around the Mediterranean and the Far East, and evinces with oral and genital ulcerations, skin lesions and uveitis. Carbonic anhydrase (CA) is a metalloenzyme which is widely distributed in the living world, and it is essential for the regulation of acid-base balance. Anti-CA antibodies have been reported in many disorders, such as systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, endometriosis, idiopathic chronic pancreatitis, type 1 diabetes and Graves' disease...
December 3, 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28162159/-the-value-of-different-antibodies-detection-in-diagnosis-of-rheumatism-with-uveitis
#5
X F Xu, J Zhang, L Cui, Y H Wang, Y Yue, L Chi, J Bai, H M Li, X X Lu
Objective: To investigate the value of HLA-B27 antigen, antinuclear antibody (ANA), anti-dsDNA and Anti-Neutrophil Cytoplasmic Antibodies (ANCA) detection in diagnosis of rheumatism with uveitis. Methods: Seven hundred and twenty four newly diagnosed patients with non-infection uveitis in Beijing Tongren Hospital from March 2012 to March 2016 who long-term lived in Beijing and its surrounding areas were continuously enrolled. HLA-B27 antigen expressions in peripheral blood lymphocytes and ANA, anti-dsDNA and ANCA levels in serum were tested...
January 24, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28129926/panniculitis-a-summary
#6
Mark R Wick
The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda...
December 27, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28088958/-clinical-effect-analysis-of-endovascular-repair-for-aortic-pseudoaneurysms-in-13-cases
#7
B L Wang, R Yang, Jll Wang, Y D Jia, G T Wang
Objective: To discuss the clinical safety and efficacyof endovascular aneurysm repair (EVAR) for aortic pseudoaneurysms. Methods: From October 2008 to October 2015, 13 patients (11 male, 2 female, with a mean age of 55.6) with aortic pseudoaneurysms treated by EVAR wereenrolled. All the 13 casesunderwentcomputed tomographic arteriography (CTA). The etiology diagnosis withdescendingaortic pseudoaneurysms, infected abdominal aortic pseudoaneurysms, abdominal aortic pseudoaneurysmsin Behcet's syndrome, and uncertain reasons were 4, 4, 4, and 1 case, respectively...
January 10, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28035223/serum-ghrelin-levels-in-patients-with-behcet-s-disease
#8
Ilker Erden, Haydar Uçak, Betül Demir, Demet Cicek, Selma Bakar Dertlioğlu, Suleyman Aydin, Savas Ozturk
INTRODUCTION: Behcet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology. AIM: To measure serum ghrelin levels in BD patients and healthy controls and to investigate its association with metabolic syndrome (MetS). MATERIAL AND METHODS: Thirty BD patients and 30 healthy individuals were enrolled in the study. Ghrelin levels were measured in blood samples using ELISA. RESULTS: The mean serum ghrelin level in BD patients (28...
December 2016: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28032234/the-emerging-role-of-interleukin-il-1-in-the-pathogenesis-and-treatment-of-inflammatory-and-degenerative-eye-diseases
#9
Claudia Fabiani, Jurgen Sota, Gian Marco Tosi, Rossella Franceschini, Bruno Frediani, Mauro Galeazzi, Donato Rigante, Luca Cantarini
Interleukin (IL)-1 plays a key role in the pathogenesis and thereafter in the search for specific treatments of different inflammatory and degenerative eye diseases. Indeed, an overactivity of IL-1 might be an initiating factor for many immunopathologic sceneries in the eye, as proven by the efficacy of the specific IL-1 blockade in different ocular diseases. For instance, the uveitis in monogenic autoinflammatory disorders, such as Blau syndrome and cryopyrin-associated periodic syndrome, or in complex polygenic autoinflammatory disorders, such as Behçet's disease, has been successfully treated with IL-1 blockers...
December 28, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27987350/influence-of-beh%C3%A3-et-s-disease-on-first-and-second-trimester-serum-screening-markers
#10
Zehra Vural Yılmaz, Gülenay Gençosmanoğlu Türkmen, Elif Yılmaz, Korkut Dağlar, Ayşe Kırbaş, Cem Sanhal, Aykan Yücel, Dilek Uygur
AIM: Behçet's disease (BD) is a rare and multisystemic vasculitis disease. In this study, we investigated whether BD had any effect on the biochemical components of first and second trimester aneuploidy screening tests. METHODS: A case-control retrospective study was conducted with 32 pregnant women with BD and 60 healthy pregnant women as controls. All pregnant womens' first trimester maternal serum pregnancy-associated plasma protein-A, free β-human chorionic gonadotropin and second trimester serum alpha-fetoprotein, unconjugated estriol and total human chorionic gonadotropin levels were examined from medical records...
December 17, 2016: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/27966293/beh%C3%A3-et-syndrome-the-vascular-cluster
#11
Hasan Yazıcı, Emire Seyahi
Although skin-mucosa lesions are common in almost all patients with Behçet syndrome (BS), clinical properties may differ from one patient to another. Within BS, there are subsets with different organ involvement and hence probably different pathological pathways. These subsets can be described as a) solo skin-mucosa disease with no major organ involvement, b) eye disease, c) seronegative spondyloarthropathy-like disease (arthritis, enthesopathy, and folliculitis), d) Crohn-like disease, and finally the topic of this chapter: e) vascular disease...
November 17, 2016: Turkish Journal of Medical Sciences
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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