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Behcets syndrome

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https://www.readbyqxmd.com/read/29775082/profile-of-renal-aa-amyloidosis-in-older-and-younger-individuals-a-single-centre-experience
#1
Siyar Erdogmus, Zeynep Kendi Celebi, Serkan Akturk, Gizem Kumru, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Kenan Keven
OBJECTIVE: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis...
May 18, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29772313/treatment-of-the-median-arcuate-ligament-syndrome-in-a-patient-with-behcet-s-disease-median-arcuate-ligament-syndrome-accompanying-to-behcet-s-disease
#2
Serkan Burç Deşer, Savas Yuruker, Mustafa Kemal Demirag
Compression of the celiac artery by the median arcuate ligament (MAL) and neurofibrous tissue is called MAL syndrome or Dunbar's syndrome. Postprandial abdominal pain, weight loss are the main symptoms. Surgical treatment still remains the gold standard therapy. Here, we present the first case to our knowledge that accompanying Behcet's disease to Median Arcuate Ligament Syndrome.
May 14, 2018: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29736629/hemoptysis-in-beh%C3%A3-et-s-syndrome-from-bedside-to-bench
#3
Giovanni Maria Rossi, Giacomo Emmi, Augusto Vaglio
No abstract text is available yet for this article.
May 7, 2018: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/29704959/associated-arterial-and-venous-cerebral-manifestations-in-beh%C3%A3-et-s-disease
#4
C Brenière, C Blanc, H Devilliers, M Samson, B Delpont, P Bielefeld, J-F Besancenot, M Giroud, Y Béjot
Behçet's disease is a rare multisystemic vasculitis with an etiology that is still unknown. Neurological manifestations may be seen in approximately 5-15% of patients, and both parenchymal and extraparenchymal neurological involvement has been described. When cerebral venous thrombosis (CVT) is the main extraparenchymal manifestation of Behçet's disease, the condition is then dubbed "angio-Behçet's syndrome". However, arterial involvement is extremely rare, with only one reported case of vasculo-neuro-Behçet's disease, characterized by both venous and intracranial arterial involvement - until now...
April 25, 2018: Revue Neurologique
https://www.readbyqxmd.com/read/29682107/what-is-vascular-beh%C3%A3-et-s-disease
#5
REVIEW
Hiroyuki Ishibashi
Vascular Behçet's disease (BD) would keep risk of anastomotic pseudoaneurysm due to deterioration of the disease even after vascular surgery was successfully done. Therefore, it is one of the least-welcome diseases for vascular surgeons. There still exist several points on a concept and criteria of the vascular BD which not only general practitioners but also the vascular surgeons do not understand. Clinical findings strongly suspecting vascular BD are follows; saccular aneurysms without atherosclerosis developed in younger than 50-year-old patients, superior vena cava syndrome or deep vein thrombosis in bilateral legs without apparent causes, and multiple superficial thrombophlebitis, etc...
March 25, 2018: Annals of Vascular Diseases
https://www.readbyqxmd.com/read/29676522/adalimumab-based-treatment-versus-dmards-for-venous-thrombosis-in-beh%C3%A3-et-syndrome-a-retrospective-study-of-70-patients-with-vascular-involvement
#6
Giacomo Emmi, Antonio Vitale, Elena Silvestri, Maria Boddi, Matteo Becatti, Claudia Fiorillo, Claudia Fabiani, Bruno Frediani, Lorenzo Emmi, Gerardo Di Scala, Matteo Goldoni, Alessandra Bettiol, Augusto Vaglio, Luca Cantarini, Domenico Prisco
OBJECTIVES: Since Behçet syndrome (BS) is the prototype of inflammation-induced thrombosis, immunosuppressants are recommended in place of anticoagulants. Here we assessed the clinical efficacy and the corticosteroid-sparing effect of adalimumab (ADA)-based treatment versus DMARDs in a large retrospective cohort of patients with BS-related venous thrombosis. METHODS: We retrospectively collected data from 70 BS patients treated with DMARDs or ADA-based regimens (ADA ± DMARDs) because of venous complications...
April 20, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29653210/neutrophilic-dermatoses-part-i-pathogenesis-sweet-syndrome-neutrophilic-eccrine-hidradenitis-and-beh%C3%A3-et-s-disease
#7
REVIEW
Caroline A Nelson, Sasha Stephen, Hovik J Ashchyan, William D James, Robert G Micheletti, Misha Rosenbach
Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features, but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes...
April 10, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29625968/2018-update-of-the-eular-recommendations-for-the-management-of-beh%C3%A3-et-s-syndrome
#8
Gulen Hatemi, Robin Christensen, Dongsik Bang, Bahram Bodaghi, Aykut Ferhat Celik, Farida Fortune, Julien Gaudric, Ahmet Gul, Ina Kötter, Pietro Leccese, Alfred Mahr, Robert Moots, Yesim Ozguler, Jutta Richter, David Saadoun, Carlo Salvarani, Francesco Scuderi, Petros P Sfikakis, Aksel Siva, Miles Stanford, Ilknur Tugal-Tutkun, Richard West, Sebahattin Yurdakul, Ignazio Olivieri, Hasan Yazici
Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search...
June 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29623390/the-role-of-genetics-and-epigenetics-in-rheumatic-diseases-are-they-really-a-target-to-be-aimed-at
#9
REVIEW
Masaru Kato, Shinsuke Yasuda, Tatsuya Atsumi
To date, numerous genetic and epigenetic studies have been performed and provided a crucial step forward in our understanding of the pathogenesis of rheumatic diseases. However, most of the recent advances in the treatment of rheumatic diseases including biological therapies are not based on or even discrepant from these genetic and epigenetic findings. For example, tumor necrosis factor inhibitors are quite successful in the treatment of rheumatoid arthritis (RA), Behçet's disease (BD), ankylosing spondylitis (AS) and psoriatic arthritis (PsA) but not in that of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren's syndrome (SS) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV), conversely, RA shares genetic backgrounds more with SLE, SSc, SS and AAV than BD, AS and PsA...
April 5, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29605630/-99m-tc-hmpao-brain-spect-in-the-monitoring-of-cerebral-vasculitis-therapy
#10
V Frantellizzi, M Morreale, M Pontico, A Francia, F M Drudi, A Farcomeni, M Liberatore
OBJECTIVE: The central nervous system (CNS) may be involved in a variety of inflammatory diseases of the blood vessels, generally known as vasculitis. The clinical diagnosis of such involvement in early stages is difficult, since a mild cognitive impairment can be the only symptom. It was hypothesized that brain-perfusion SPECT would be able to reveal CNS involvement and to monitor the course of the disease. The purpose of this study was assess if and when an improvement of cerebral perfusion can be registered by SPECT during the follow-up of these diseases...
March 28, 2018: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/29600965/considerations-in-designing-and-interpreting-prevalence-studies-for-beh%C3%A3-et-syndrome
#11
Gulen Hatemi, Sinem Nihal Esatoglu, Sebahattin Yurdakul
No abstract text is available yet for this article.
March 29, 2018: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29561414/behcet-disease-combined-with-sjogren-syndrome-a-unique-case-report-and-literature-review
#12
Fang-He Ju, Ting-Zhen Xu, Hui-Hua Hong, He Mao, Meng Wang, Zhen Wang
RATIONALE: Behcet disease(BD) and Sjogren syndrome(SS) are separate conditions that rarely concomitantly affect an individual. In theory,mild symptoms of patients with BD or SS are easy to igore and,thus,remain undiagnosed. There,it is reasonable to believe there may be some clinical cases of combined diseases that go undiscovered and which needs to be taken seriously. In addition,it has been suggested that herpes simplex virus(HSV) types 1 and 2 are associated with BD,but have not been shown to be correlated to the direct pathogenesis of BD...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29545422/myelodysplastic-syndrome-presenting-as-a-beh%C3%A3-et-s-like-disease-with-aortitis
#13
Shudan Wang, Noam Broder, Paula Marchetta, Johannes Nowatzky
A 46-year-old Hispanic man presented with fever, genital ulcers, left eye redness and chest pain. Physical examination was notable for a healed oral ulcer and scrotal ulcers, and bilateral superficial thrombophlebitis. He was found to have new-onset pancytopenia. CT of the chest showed pericardial and pleural effusions and rapidly progressing inflammation of the aortic arch and ascending vessels. Although the patient had Behcet's disease (BD)-like symptoms, pancytopenia could not be explained by the diagnosis, prompting a bone marrow biopsy which showed myelodysplastic syndrome...
March 15, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29490685/a-novel-single-variant-in-the-mefv-gene-causing-mediterranean-fever-and-beh%C3%A3-et-s-disease-a-case-report
#14
Maria Zerkaoui, Fatima Zahra Laarabi, Yousra Ajhoun, Bouchra Chkirate, Abdelaziz Sefiani
BACKGROUND: Familial Mediterranean fever is an autoinflammatory disease of unknown etiology, characterized clinically by recurrent attacks of sudden-onset fever with arthralgia and/or thoracoabdominal pain and pathogenetically by autosomal recessive inheritance due to a mutation in the MEFV gene. Behçet's disease is an inflammatory disease characterized by recurrent oral and genital aphthous ulcerations, uveitis, and skin lesions. Preliminarily, our literature review suggested that patients with familial Mediterranean fever who also have Behçet's disease have only a single mutated familial Mediterranean fever gene...
March 1, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29481922/recurrent-iliofemoral-venous-thrombosis-in-the-setting-of-may-thurner-syndrome-as-the-presenting-symptom-of-behcet-s-disease
#15
Sameer Lakha, Chien Yi Maximilian Png, Kevin Chun, Windsor Ting
BACKGROUND: Vascular manifestations including pulmonary artery aneurysms and venous thrombosis are seen in up to 14% of patients with Behcet's disease. We report a patient who had recurrent deep vein thrombosis (DVT) as the presenting symptom of Behcet's Disease. METHODS: A 19-year-old male who presented with acute iliofemoral DVT, confirmed by intravascular ultrasound (IVUS) and venogram. May-Thurner syndrome was also observed. Repeated catheter-based pharmacomechanical thrombolysis, thrombectomy, and subsequent iliac vein stenting were performed...
May 2018: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29430432/hughes-stovin-syndrome-as-an-outcome-of-beh%C3%A3-et-disease-or-as-a-different-entity
#16
Serkan Demirkan, Yıldırım Gültekin
Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease...
February 2018: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29429154/cancer-risk-in-patients-with-intestinal-beh%C3%A3-et-s-disease-a-nationwide-population-based-study
#17
Minkyung Han, Yoon Suk Jung, Won Ho Kim, Jae Hee Cheon, Sohee Park
Background/Aims: The relationship between intestinal Behçet's disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with intestinal BD. Methods: Using the National Health Insurance claims records, we collected data on 365 patients who had been diagnosed with intestinal BD between 2011 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with intestinal BD in comparison with the general population were calculated...
February 13, 2018: Gut and Liver
https://www.readbyqxmd.com/read/29390339/infliximab-associated-with-life-threatening-lung-infection-in-a-patient-with-behcet-disease-with-intestinal-and-hematopoietic-system-involvement-a-case-report
#18
Yong Chen, Yan Shen, Hai-Fen Ma, Jian-Fei Cai, Yan-Qin Hua, Jun Zou, Jian-Long Guan
RATIONALE: Tumor necrosis factor (TNF-α) participates in the pathophysiology of Behcet's disease (BD) and myelodysplastic syndrome (MDS). Infliximab is recommaned for the most severe type of BD, however, there is little evidence for its effectiveness in BD associated MDS. PATIENT CONCERNS: A 46-year-old female, initially diagnosed with intestinal BD and leukopenia was later diagnosed as MDS. Treatement with infliximab and other immunoregulators lead to life-threatening pneumonia...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29377079/incidence-of-diseases-primarily-affecting-the-skin-by-age-group-population-based-epidemiologic-study-in-olmsted-county-minnesota-and-comparison-with-age-specific-incidence-rates-worldwide
#19
REVIEW
Laurel L Wessman, Louise K Andersen, Mark D P Davis
Understanding the effects of age on the epidemiology of diseases primarily affecting the skin is important to the practice of dermatology, both for proper allocation of resources and for optimal patient-centered care. To fully appreciate the effect that age may have on the population-based calculations of incidence of diseases primarily affecting the skin in Olmsted County, Minnesota, and worldwide, we performed a review of all relevant Rochester Epidemiology Project-published data and compared them to similar reports in the worldwide English literature...
January 29, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29362466/beh%C3%A3-et-syndrome-a-contemporary-view
#20
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
This corrects the article DOI: 10.1038/nrrheum.2017.208.
January 24, 2018: Nature Reviews. Rheumatology
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