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juvenile Behcet's disease

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https://www.readbyqxmd.com/read/27858841/marshall-syndrome-in-a-young-child-a-reality-case-report
#1
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/27698552/profile-of-adalimumab-and-its-potential-in-the-treatment-of-uveitis
#2
Stephen J Balevic, C Egla Rabinovich
Uveitis refers to the presence of intraocular inflammation, and as a strict definition compromises the iris and ciliary body anteriorly and the choroid posteriorly (the uvea). Untreated, uveitis can lead to visual loss or blindness. The etiology of uveitis can include both infectious and noninfectious (usually immune-mediated) causes, the latter of which are often mediated predominantly by Th1 CD4(+) T-cells that secrete proinflammatory cytokines. Tumor necrosis factor-alpha (TNF-α) is a proinflammatory cytokine involved in the pathogenesis of uveitis, which at high concentrations can cause excess inflammation and tissue damage...
2016: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/27631190/a-comprehensive-proteomics-analysis-of-the-human-iris-tissue-ready-to-embrace-postgenomics-precision-medicine-in-ophthalmology
#3
Krishna R Murthy, Manjunath Dammalli, Sneha M Pinto, Kalpana Babu Murthy, Raja Sekhar Nirujogi, Anil K Madugundu, Gourav Dey, Yashwanth Subbannayya, Uttam Kumar Mishra, Bipin Nair, Harsha Gowda, T S Keshava Prasad
The annual economic burden of visual disorders in the United States was estimated at $139 billion. Ophthalmology is therefore one of the salient application fields of postgenomics biotechnologies such as proteomics in the pursuit of global precision medicine. Interestingly, the protein composition of the human iris tissue still remains largely unexplored. In this context, the uveal tract constitutes the vascular middle coat of the eye and is formed by the choroid, ciliary body, and iris. The iris forms the anterior most part of the uvea...
September 2016: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/27515671/the-use-of-cyclosporine-a-in-rheumatology-a-2016-comprehensive-review
#4
Cecilia Beatrice Chighizola, Voon H Ong, Pier Luigi Meroni
Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease...
August 11, 2016: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/27420799/recurrent-bilateral-anterior-uveitis-with-kikuchi-fujimoto-disease
#5
Marta Gómez-Mariscal, Andrea Oleñik, Pablo Arriba-Palomero, Mónica García-Cosio, Francisco J Muñoz-Negrete
PURPOSE: To report an uncommon case of Kikuchi-Fujimoto disease-associated-uveitis. METHODS: A 31-year-old Caucasian woman with cervical lymphadenitis and Kikuchi-Fujimoto disease recently confirmed by biopsy, complained about unilateral blurry vision. Differential diagnoses, including non-Hodgkin lymphoma, tuberculosis, sarcoidosis, Behçet disease, rheumatoid arthritis, and juvenile idiopathic arthritis was performed. We undertook a review of other similar cases found in the literature...
July 15, 2016: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/27402112/juvenile-spondyloarthropathies
#6
REVIEW
Amra Adrovic, Kenan Barut, Sezgin Sahin, Ozgur Kasapcopur
Juvenile spondyloarthropathies represent a clinical entity separate from the adult disease. Initial clinical signs of juvenile spondyloarthropathies often include lower extremity arthritis and enthesopathy, without axial involvement at the disease onset. Asymmetrical oligoarthritis of lower extremities is typically seen in this type of arthritis. Enthesopathy, which is the hallmark of the disease, is most commonly seen in the Achilles tendon, being manifested by heel pain. Anterior uveitis and HLA-B27 positivity are seen in a proportion of cases...
August 2016: Current Rheumatology Reports
https://www.readbyqxmd.com/read/27319272/-peripheral-artery-disease-in-patients-younger-than-50%C3%A2-years-old-which-etiology
#7
S Cotard, A Nouni, V Jaquinandi, G Gladu, A Kaladji, G Mahé
Peripheral arterial disease (PAD) encompasses disease of all arteries of the body except the coronary arteries. The main etiology whatever the patient's age is atherosclerosis. Different etiologies can induce PAD especially when patients are younger than 50 years old and have no cardiovascular risk factors (smoking, hypertension, diabetes…). PAD that appears before 50 years old can be named juvenile PAD (JPAD) although there is no consensus about the definition. The aim of this work is to present the different etiologies of JPAD according to their hereditary, acquired or mixed origins...
September 2016: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/27100417/characteristics-treatment-and-long-term-outcome-of-gastrointestinal-involvement-in-behcet-s-syndrome-a-strobe-compliant-observational-study-from-a-dedicated-multidisciplinary-center
#8
Ibrahim Hatemi, Sinem Nihal Esatoglu, Gulen Hatemi, Yusuf Erzin, Hasan Yazici, Aykut Ferhat Celik
Gastrointestinal involvement is rare in Behçet's syndrome (BS) patients from the Mediterranean basin. We report the demographic and disease characteristics, treatment modalities, and outcome of patients with gastrointestinal involvement in BS (GIBS). We retrospectively reviewed the charts of all BS patients in our BS clinic with a diagnosis of GIBS. Patients were invited to the clinic to assess their outcome. Among 8763 BS patients, we identified 60 with GIBS (M/F: 32/28, mean age at diagnosis: 34 ± 10, mean follow-up: 7...
April 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27055672/development-and-validation-of-a-bayesian-network-for-the-differential-diagnosis-of-anterior-uveitis
#9
J J González-López, Á M García-Aparicio, D Sánchez-Ponce, N Muñoz-Sanz, N Fernandez-Ledo, P Beneyto, M C Westcott
PurposeTo develop and validate a Bayesian belief network algorithm for the differential diagnosis of anterior uveitis.Patients and methodsThe 11 most common etiologies were included (idiopathic, ankylosing spondylitis, psoriasic arthritis, reactive arthritis, inflammatory bowel diseases, sarcoidosis, tuberculosis, Behçet, Posner-Schlossman syndrome, juvenile idiopathic arthritis (JIA), and Fuchs' heterochromic cyclitis). Frequencies of association between factors and etiologies were retrieved from a systematic review of the literature...
June 2016: Eye
https://www.readbyqxmd.com/read/27015607/infliximab-versus-adalimumab-in-the-treatment-of-refractory-inflammatory-uveitis-a-multicenter-study-from-the-french-uveitis-network
#10
Hélène Vallet, Pascal Seve, Lucie Biard, Jean Baptiste Fraison, Philip Bielefeld, Laurent Perard, Boris Bienvenu, Sébastien Abad, Aude Rigolet, Alban Deroux, Damien Sene, Antoinette Perlat, Isabelle Marie, Elodie Feurer, Eric Hachulla, Olivier Fain, Gaëlle Clavel, Sophie Riviere, Pierre-Alban Bouche, Julie Gueudry, Gregory Pugnet, Phuc Le Hoang, Matthieu Resche Rigon, Patrice Cacoub, Bahram Bodaghi, David Saadoun
OBJECTIVE: To analyze the factors associated with response to anti-tumor necrosis factor (anti-TNF) treatment and compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in patients with refractory noninfectious uveitis. METHODS: This was a multicenter observational study of 160 patients (39% men and 61% women; median age 31 years [interquartile range 21-42]) with uveitis that had been refractory to other therapies, who were treated with anti-TNF (IFX 5 mg/kg at weeks 0, 2, 6, and then every 5-6 weeks [n = 98] or ADA 40 mg every 2 weeks [n = 62])...
June 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/26940286/safety-profile-of-anakinra-in-the-management-of-rheumatologic-metabolic-and-autoinflammatory-disorders
#11
REVIEW
Giuseppe Lopalco, Donato Rigante, Margherita Giannini, Mauro Galeazzi, Giovanni Lapadula, Florenzo Iannone, Luca Cantarini
Anakinra is a biologic response modifier that competitively antagonises the biologic effects of interleukin-1, the ancestor pleiotropic proinflammatory cytokine produced by numerous cell types, found in excess in the serum, synovial fluid and any involved tissues of patients with many inflammatory diseases. The magnitude of the risk of different infections, including Mycobacterium tuberculosis (Mtb) infection, associated with the large use of anakinra in many rheumatologic, metabolic or autoinflammatory disorders is still unknown...
May 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/26933204/autoimmunity-including-intestinal-beh%C3%A3-et-disease-bearing-the-kras-mutation-in-lymphocytes-a-case-report
#12
Hiroshi Moritake, Masatoshi Takagi, Mariko Kinoshita, Osamu Ohara, Shojiro Yamamoto, Sayaka Moriguchi, Hiroyuki Nunoi
We experienced the case of a 3-year-old male with a very rare combination of autoimmunity, including immune thrombocytopenia, recurrent Henoch-Schönlein purpura and intestinal Behçet disease. Exome sequencing of the patient's peripheral blood mononuclear cells identified a KRAS G13C mutation. Interestingly, the KRAS G13C mutation was observed in T and B lymphocytes, as well as natural killer cells, but not granulocytes. Our case was completely phenotypically different from RASopathies and did not meet the criteria for Ras-associated lymphoproliferative disease or juvenile myelomonocytic leukemia...
March 2016: Pediatrics
https://www.readbyqxmd.com/read/26849760/-hodgkin-s-lymphoma-and-autoimmunity-is-there-a-relationship
#13
Mónica Jerónimo, Sónia Silva, Manuela Benedito, Manuel João Brito
INTRODUCTION: The relationship between lymphomas and autoimmune diseases has been reported as bi-directional, however there is a few data in pediatric population. The aim of this work is to evaluate the prevalence of autoimmune diseases in children and adolescents with Hodgkin's lymphoma followed in a Pediatric Oncology Unit. MATERIAL AND METHODS: By reviewing Hodgkin's lymphomas data from the past 16 years (collected prospectively), an apparently large incidence of autoimmune diseases, mostly in female patients, was noted...
November 2015: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/26736030/juvenile-adamantiades-beh%C3%A3-et-disease
#14
Aristeidis G Vaiopoulos, Meletios A Kanakis, Violetta Kapsimali, Georgios Vaiopoulos, Phedon G Kaklamanis, Christos C Zouboulis
Adamantiades-Behçet disease (ABD) is a chronic, multisystemic, recurrent, inflammatory vascular disorder of unknown etiology. Patients with symptoms initially appearing at the age of 16 or less are considered as cases of juvenile-onset ABD (JABD). JABD is relatively rare compared to ABD of adults, and only case reports and case studies have been published regarding this subtype of the disease. Epidemiology, clinical features, diagnosis and treatment of JABD are discussed in this review.
2016: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/26706401/clinical-manifestations-in-uveitis-patients-with-and-without-rheumatic-disease-in-a-chinese-population-in-taiwan
#15
Shi-Ting Tseng, Tsung-Chieh Yao, Jing-Long Huang, Kuo-Wei Yeh, Yih-Shiou Hwang
BACKGROUND/PURPOSE: Uveitis can be a local eye disease or a manifestation of systemic rheumatologic disorders. However, the differences of clinical manifestations between uveitis patients with or without systemic rheumatologic disease have been seldom described in literature. We investigated the clinical features and complications of rheumatic disease-related uveitis, and compared the characteristics in patients with and without rheumatic disease in a Chinese population in Taiwan. METHODS: A retrospective review was performed for all patients who had been diagnosed with uveitis between January 2009 and June 2014 at the Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan...
November 17, 2015: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/26609459/efficacy-of-adalimumab-in-a-girl-with-refractory-intestinal-behcet-s-disease
#16
Mariko Kaji, Takayuki Kishi, Takako Miyamae, Satoru Nagata, Hisashi Yamanaka, Satoshi Fujikawa
We describe our experience with a juvenile patient who had refractory intestinal Behcet's disease that responded to adalimumab, a fully humanized antibody against soluble TNF-α and its receptor. The patient, a 13-year-old girl, presented with oral aphthous ulcers, vulvar pain, and rashes on the lower extremities. She gradually developed a low-grade fever, abdominal pain, diarrhea, and hematochezia. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum, consistent with intestinal Behcet's disease...
2015: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/26237061/recurrent-fever-of-unknown-origin-fuo-due-to-periodic-fever-aphthous-stomatititis-pharyngitis-and-adenitis-fapa-syndrome-in-an-adult
#17
Sigridh Muñoz-Gómez, Burke A Cunha
FAPA syndrome (periodic fever, aphthous stomatititis, pharyngitis and adenitis) is a relatively new entity described in pediatric patients. In adults, reports of FAPA are limited to rare case reports. The differential diagnosis of FAPA in adults includes Behcet's syndrome, familial Mediterranean fever (FMF), Hyper IgD syndrome and juvenile rheumatoid arthritis (JRA), i.e., adult Still's disease. With FAPA syndrome, between episodes patients are completely asymptomatic and serologic inflammatory markers such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and white blood cell (WBC) count are normal...
2013: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/26023856/a-new-alternative-therapy-in-dermatology-tocilizumab
#18
Özgül Muştu Koryürek, Göknur Kalkan
Tocilizumab (TCZ) is a recombinant-humanized anti-human interleukin 6 receptor monoclonal antibody of the immunoglobulin (Ig) IgG1 subclass with a H2L2 polypeptide structure. Even if it was approved by the US Food and Drug Administration for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis and polyarticular juvenile idiopathic arthritis, satisfying results have also been reported with TCZ in various refractory dermatological diseases such as psoriasis, psoriatic arthritis, Behçet's disease, systemic lupus erythematosus, systemic sclerosis, relapsing polychondritis, vasculitis and atopic dermatitis...
2016: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/25247023/demographical-and-clinical-characteristics-of-behcet-s-disease-in-southeastern-turkey
#19
Bilal Sula, Ibrahim Batmaz, Derya Ucmak, Ilyas Yolbas, Sedat Akdeniz
BACKGROUND: We aimed to determine the demographic and clinical features of patients with Behcet's disease (BD) in Southeastern Turkey. METHODS: In this study, files of 132 patients with BD (76 females and 56 males) who were diagnosed with BD according to the International Study Group criteria at the Department of Dermatology of Dicle University Faculty of Medicine from 2005 to 2009 were evaluated retrospectively. Demographical and clinical characteristics of the cases were recorded...
December 2014: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/24774505/adalimumab-in-the-treatment-of-immune-mediated-diseases
#20
REVIEW
G Lapadula, A Marchesoni, A Armuzzi, C Blandizzi, R Caporali, S Chimenti, R Cimaz, L Cimino, P Gionchetti, G Girolomoni, P Lionetti, A Marcellusi, F S Mennini, C Salvarani
Tumour necrosis factor (TNF) plays an important role in the pathogenesis of immune-mediated inflammatory diseases (IMIDs). TNF inhibition results in down-regulation of abnormal and progressive inflammatory processes, resulting in rapid and sustained clinical remission, improved quality of life and prevention of target organ damage. Adalimumab is the first fully human monoclonal antibody directed against TNF. In this article, we review the role and cost effectiveness of adalimumab in the treatment of IMIDs in adults and children...
January 2014: International Journal of Immunopathology and Pharmacology
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