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    High-dose intravenous steroid pulse therapy in ocular involvement of Behcet's disease: a pilot double-blind control study.
    Int J Rheum Dis 2017 May 19. Epub 2017 May 19.
    Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran, Iran.
    Aim: To evaluate the efficacy of intravenous high-dose pulses of methylprednisolone (IVPM) for treatment of ocular involvement in Behcet's disease (BD).

    Method: In a double-blind control study, we randomized BD patients with posterior uveitis (PU) and/or retinal vasculitis (RV) into two groups. They received either IVPM (1000 mg methylprednisolone) or placebo for 3 consecutive days. Read More

    Infantile-onset thiamine responsive megaloblastic anemia syndrome with SLC19A2 mutation: a case report.
    Arch Argent Pediatr 2017 06;115(3):e153-e156
    Department of Pediatric Endocrinology, Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
    Background: Thiamine-responsive megaloblastic anemia syndrome (TRMA), also known as Rogers syndrome, is characterized by megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Disturbances of the thiamine transport into the cells results from homozygous or compound heterozygous mutations in the SLC19A2 gene.

    Case Presentation: We report a girl which presented with sensorineural deafness treated with a hearing prosthesis, insulin requiring diabetes, macrocytic anemia, treated with thiamine (100 mg/day). Read More

    Successful Treatment of Behçet's Disease Associated with Acute Myeloid Leukemia with Myelodysplasia-related Changes Using Azacitidine and Tacrolimus before Allogeneic Hematopoietic Stem Cell Transplantation.
    Intern Med 2017 15;56(10):1199-1202. Epub 2017 May 15.
    Third Department of Internal Medicine, Yamaguchi University School of Medicine, Japan.
    The coexistence of acute myeloid leukemia (AML) with Behçet's disease (BD) is rare. The optimum treatment for AML-associated BD has not been established. We herein report a patient with BD who developed AML with myelodysplasia-related changes. Read More

    Ustekinumab for Behçet's disease.
    J Autoimmun 2017 May 5. Epub 2017 May 5.
    Sorbonne Universités, UPMC Université Paris 06, UMR 7211, Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biotherapie (DHU i2B), F-75005, Paris, France; INSERM, UMR_S 959, F-75013, Paris, France; CNRS, FRE3632, F-75005, Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Département de Médecine Interne et Immunologie Clinique, F-75013, Paris, France. Electronic address:
    Objective: To evaluate the efficacy and safety of ustekinumab in the treatment of oral ulcers (OU) in patients with Behçet's disease (BD).

    Patients And Methods: Prospective study including 14 patients [median age of 39 (34; 41) years, with 71% of men] fulfilling criteria of the International Study Group for BD and with active OU resistant to colchicine. Patients received ustekinumab 90 mg (n = 11) or 45 mg (n = 3) subcutaneously at inclusion, at week 4, and every 12 weeks. Read More

    [Clinical analysis of Behçet disease associated with pulmonary vascular disease].
    Zhonghua Jie He He Hu Xi Za Zhi 2017 May;40(5):343-348
    Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
    Objective: To investigate the clinical features, therapy and prognosis of Behcet's disease(BD) complicated with pulmonary vascular diseases (PVD). Methods: The clinical manifestation, hemodynamics, pulmonary artery angiographic findings, therapy and prognosis of PVD in BD in Shanghai Pulmonary Hospital from January 2009 to August 2016 were analyzed retrospectively. Results: Seven patients with average of (37±20) years were included. Read More

    Atrium Advanta V12 large diameter stent-graft applications in infra-renal abdominal aortic pseudo-aneurysms (due to Behcet disease): Safety and efficacy.
    Ann Vasc Surg 2017 May 4. Epub 2017 May 4.
    Osmangazi University, Faculty of Medicine, Department of Interventional Radiology, Eskisehir/TURKEY. Electronic address:
    Purpose: To determine the safety and effectiveness of Atrium Advanta V12 large diameter stent-graft applications for infra-renal abdominal aortic pseudoaneurysms (due to Behcet disease).

    Method: Data of Advanta V12™ (Atrium Europe B.V, Mijdrecht, the Netherlands) applied 12 female patients (mean age 30. Read More

    Retrospective Study Evaluating Treatment Decisions and Outcomes of Childhood Uveitis Not Associated with Juvenile Idiopathic Arthritis.
    J Pediatr 2017 Apr 27. Epub 2017 Apr 27.
    Pediatric Rheumatology, Centre de Référence des Maladies Auto-Inflammatoires, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Paris-Sud University Hospital, Le Kremlin-Bicêtre, France.
    Objective: To evaluate treatment, ocular complications and outcomes of children with pediatric uveitis not associated with juvenile idiopathic arthritis.

    Study Design: This was a retrospective chart review of pediatric uveitis in children under 16 years of age, recruited from the pediatric rheumatology department at Bicêtre Hospital from 2005 to 2015. Patients with juvenile idiopathic arthritis-associated and infectious uveitis were excluded. Read More

    Evaluation of Optic Nerve with Strain and Shear Wave Elastography in Patients with Behçet's Disease and Healthy Subjects.
    Ultrasound Med Biol 2017 Jul 24;43(7):1348-1354. Epub 2017 Apr 24.
    Department of Ophtalmology, Faculty of Medicine, Kirikkale University, Yahsihan, Kirikkale, Turkey.
    The objective of this study was to investigate the elasticity characteristics of the optic nerve using strain and shear wave elastography in patients with Behçet's disease and to compare the results with those of healthy volunteers. Forty-six optic nerves from patients with Behçet's disease and 54 optic nerves from healthy volunteers were investigated prospectively in this study using strain and shear wave elastography. There was a statistically significant difference in terms of elasticity patterns between patients and healthy volunteers (p < 0. Read More

    Prevalence of fibromyalgia in general population and patients, a systematic review and meta-analysis.
    Rheumatol Int 2017 Apr 26. Epub 2017 Apr 26.
    Health Sciences Research Center, Addiction Institute, Mazandaran University of Medical Sciences, Sari, Iran.
    This study aims to estimate the reliable prevalence of fibromyalgia using meta-analysis method. Available databanks were searched using appropriate keywords. According to the heterogeneity between the results (indicated by Cochrane and I square indices), random- or fixed-effects model was applied to combine the point prevalences. Read More

    The Behçet's disease-associated variant of the aminopeptidase ERAP1 shapes a low affinity HLA-B*51 peptidome by differential subpeptidome processing.
    J Biol Chem 2017 Apr 26. Epub 2017 Apr 26.
    Centro de Biologia Molecular Severo Ochoa, Spain;
    A low activity variant of endoplasmic reticulum aminopeptidase 1 (ERAP1), Hap10, is associated with the autoinflammatory Behcet s disease (BD) in epistasis with HLA-B*51, which is the main risk factor for this disorder. The role of Hap10 in BD pathogenesis is unknown. We sought to define the effects of Hap10 on the HLA-B*51 peptidome and to distinguish these effects from those due to HLA-B*51 polymorphisms unrelated to disease. Read More

    Behcet Disease Initially Presenting as Deep Venous Thrombosis: A Case Report.
    J Pediatr Hematol Oncol 2017 Apr 21. Epub 2017 Apr 21.
    *Division of Oncology Departments of †Pediatrics §Pathology and Laboratory Medicine ¶Division of Hematology, The Children's Hospital of Philadelphia Departments of ‡Pediatrics ∥Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
    Behcet disease is a potentially life-threatening multisystemic vasculitis with thrombotic tendency. Mucocutaneous ulcers, arthritis, and uveitis are the most recognizable features, but may be absent at the time of medical evaluation. We report a case in which a 8-year old patient presented with spontaneous bilateral lower extremity deep venous thromboses, and screening for rheumatologic symptoms led to diagnosing Behcet. Read More

    Intestinal Behçet and Crohn's disease: two sides of the same coin.
    Pediatr Rheumatol Online J 2017 Apr 20;15(1):33. Epub 2017 Apr 20.
    Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Viale Consolare Valeria, 98124, Messina, Italy.
    Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary tracts). Both disorders have fluctuating courses and when gastrointestinal symptoms are prevalent, differential diagnosis can be difficult. BD involves the gastrointestinal tract in 10-15% of cases with localized lesions in the ileocecal region. Read More

    Elevated Monocyte to High-Density Lipoprotein Cholesterol Ratio and Endothelial Dysfunction in Behçet Disease.
    Angiology 2017 Jan 1:3319717704748. Epub 2017 Jan 1.
    1 Department of Cardiology, Inonu University Faculty of Medicine, Malatya, Turkey.
    Behçet disease (BD) is a multisystemic disorder characterized by endothelial dysfunction and inflammation. Monocyte to high-density lipoprotein cholesterol ratio (MHR) is a recently emerged indicator of inflammation and oxidative stress. Sixty patients with BD and 50 control individuals were included to investigate the relationship between MHR and endothelial dysfunction. Read More

    Investigating the role of IL-33 in the pathogenesis of Behçet's Disease.
    Acta Clin Belg 2017 Apr 17:1-5. Epub 2017 Apr 17.
    a Faculty of Medicine, Department of Internal Medicine, Division of Allergy and Immunology , Ankara University , Ankara , Turkey.
    Objectives: Behçet's disease (BD) is an inflammatory disease, characterized by oral aphthous lesions, recurrent uveitis, skin lesions, and genital ulcerations. Increased release of several cytokines may play a role in the inflammatory stages of BD. IL-33, a member of the IL-1 cytokine superfamily, plays an important role in inflammation. Read More

    Clinical significance of fibromyalgia syndrome in different rheumatic diseases: Relation to disease activity and quality of life.
    Reumatol Clin 2017 Apr 11. Epub 2017 Apr 11.
    Rheumatology Department, Faculty of Medicine, Cairo University, Egypt. Electronic address:
    Objective: To describe the frequencies of fibromyalgia syndrome (FMS) in various rheumatic diseases; rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and Behçets disease (BD) patients and to study the relation to clinical manifestations and quality of life (QoL).

    Patients And Methods: 160 patients (50 RA, 50 SLE, 30 SSc and 30 BD) and matched corresponding healthy controls were included. Disease activity was assessed using disease activity score in 28 joints (DAS28) for RA, SLE Disease Activity index (SLEDAI), modified Rodnan skin score for SSc and BD Current Activity Form (BDCAF). Read More

    Frosted branch angiitis and cerebral venous sinus thrombosis as an initial onset of neuro-Behçet's disease: a case report and review of the literature.
    J Med Case Rep 2017 Apr 15;11(1):104. Epub 2017 Apr 15.
    Department of Ophthalmology, University of Sao Paulo, Dr. Eneas Carvalho de Aguiar Avenue, Sao Paulo, 255, Brazil.
    Background: Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Read More

    Adherence to guidelines for the treatment of Behçet's syndrome in New York and Amsterdam.
    Clin Exp Rheumatol 2017 Apr 13. Epub 2017 Apr 13.
    NYU Hospital for Joint Disease, New York, NY, USA.
    Objectives: To assess adherence to published guidelines for the treatment of Behçet's syndrome (BS) in two geographic areas.

    Methods: We extracted guideline statements from the 2008 EULAR recommendations. Adherence to these statements was evaluated retrospectively in both New York (USA) and Amsterdam (The Netherlands), by reviewing records from patients fulfilling the ISG criteria. Read More

    Behçet disease in Western Algeria.
    Med Sante Trop 2017 Feb;27(1):101-104
    Service de médecine interne, CHU Tidjani Damerdji Tlemcen, 13000 Algérie.
    The aim of the current study was to describe the epidemiological and clinical features of Behçet disease in western Algeria. This observational retrospective study examined records for patients with Behçet disease seen in our department from 1990 to 2015. Of the 61 patients included in the study, 80. Read More

    Celiac disease prevalence in a large series of patients with Behcet's disease.
    Int J Rheum Dis 2017 Apr 11. Epub 2017 Apr 11.
    Department of Dermatology, Ankara Education and Research Hospital, Ankara, Turkey.
    Aim: There are common findings between Behçet's disease (BD) and celiac disease (CD) based on similar immunological pathogenesis and there is only limited data available investigating the link between these two diseases. Furthermore, documented gastrointestinal (GI) involvement with marked upper GI symptoms in BD has been rarely reported. The aim of this study was to assess the prevalence of CD and to evaluate endoscopic findings in Turkish BD patients. Read More

    Effect of the timing of dialysis initiation on left ventricular hypertrophy and ınflammation in pediatric patients.
    Pediatr Nephrol 2017 Apr 10. Epub 2017 Apr 10.
    Department of Pediatric Nephrology, Tepecik Training and Research Hospital, İzmir, Turkey.
    Background: The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis.

    Methods: The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed. Read More

    Transcription Factors Regulating Inflammatory Cytokine Production Are Differentially Expressed in Peripheral Blood Mononuclear Cells of Behçet Disease Depending on Disease Activity.
    Ann Dermatol 2017 Apr 24;29(2):173-179. Epub 2017 Mar 24.
    Department of Microbiology, Ajou University School of Medicine, Suwon, Korea.; Department of Biomedical Sciences, The Graduate School, Ajou University, Suwon, Korea.
    Background: Behçet disease (BD) is a relapsing inflammatory disease with increased production of inflammatory cytokines in peripheral blood mononuclear cells (PBMCs); however, the underlying molecular mechanisms are not well known.

    Objective: To analyze whether the differential expression of transcription factors is involved in the increased tumor necrosis factor (TNF)-α and interleukin (IL)-6 production by PBMCs of BD patients compared to healthy controls (HCs).

    Methods: Expression of transcription factors was examined by real-time reverse transcriptase-polymerase chain reaction and western blotting. Read More

    Vasculitis and Pregnancy.
    Rheum Dis Clin North Am 2017 May 15;43(2):239-247. Epub 2017 Mar 15.
    Division of Rheumatology, Duke University Medical Center, Box 3535 Trent Drive, Durham, NC 27710, USA. Electronic address:
    Vasculitis is more often a disease of women beyond their reproductive years, leaving the challenges of pregnancy management difficult to study. Pregnancy complications, including pregnancy loss and preterm birth, are higher among women with all forms of vasculitis. It seems that controlling the disease before pregnancy may improve the chances of pregnancy success. Read More

    Intestinal and neurological involvement in Behcet disease: a clinical case.
    Ital J Pediatr 2017 Apr 7;43(1):33. Epub 2017 Apr 7.
    Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy.
    Background: Behcet's disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5-10%, and gastrointestinal tract involvement in 10-15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report. Read More

    Increased serum interleukin-32 levels in patients with Behçet's disease.
    Int J Rheum Dis 2017 Apr 5. Epub 2017 Apr 5.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea.
    Aim: Interleukin (IL)-32 is known to act as a proinflammatory cytokine and is likely involved in several chronic inflammatory diseases. The aims of this study were to investigate whether serum IL-32 levels are elevated in patients with Behçet's disease (BD) and to identify the correlation between IL-32 levels and disease activity.

    Methods: We enrolled 50 patients with BD and 35 healthy controls. Read More

    Initial Trabeculectomy With Mitomycin-C for Secondary Glaucoma-associated With Uveitis in Behçet Disease Patients.
    J Glaucoma 2017 Mar 31. Epub 2017 Mar 31.
    *Department of Ophthalmology, University of Tokyo School of Medicine †Department of Ophthalmology, Japan Community Health Care Organization Tokyo Shinjuku Center, Tokyo, Japan.
    Purpose: To examine clinical outcomes following an initial trabeculectomy with mitomycin-C for secondary glaucoma associated with uveitis in Behçet disease (BD) patients.

    Design: Retrospective interventional case series.

    Patients And Methods: Twenty-two eyes in 18 patients with uveitic glaucoma (UG) associated with Behçet disease who underwent an initial trabeculectomy with mitomycin-C between January 1996 and August 2014 were retrospectively reviewed. Read More

    Developing a Core Set of Outcome Measures for Behçet Disease: Report from OMERACT 2016.
    J Rheumatol 2017 Apr 1. Epub 2017 Apr 1.
    From the Division of Rheumatology, Department of Internal Medicine, Istanbul University Cerrahpasa Faculty of Medicine; Division of Rheumatology, Marmara University Faculty of Medicine; Division of Rheumatology, Department of Internal Medicine, Istanbul University Istanbul Faculty of Medicine; Academic Hospital, Istanbul, Turkey; Division of Rheumatology and Immunology, The Ohio State University Wexner Medical Center, Columbus, Ohio; Department of Family Medicine and Community Health, University of Pennsylvania; Division of Rheumatology, Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, Pennsylvania; New York University School of Medicine, NYU Hospital for Joint Diseases, New York, USA; Department of Internal Medicine, University Paris Diderot, Paris, France. Supported in part by the Vasculitis Clinical Research Consortium (VCRC; U54 AR057319 and U01 AR5187404), part of the Rare Diseases Clinical Research Network, an initiative of the Office of Rare Diseases Research and the National Center for Advancing Translational Science. G. Hatemi, MD, Associate Professor of Medicine, Division of Rheumatology, Department of Internal Medicine, Istanbul University Cerrahpasa Faculty of Medicine; A. Meara, MD, Clinical Instructor, Division of Rheumatology and Immunology, The Ohio State University Wexner Medical Center; Y. Ozguler, MD, Fellow in Rheumatology, Division of Rheumatology, Istanbul University Cerrahpasa Faculty of Medicine; H. Direskeneli, MD, Professor of Rheumatology, Division of Rheumatology, Marmara University Faculty of Medicine; A. Mahr, MD, PhD, Professor of Internal Medicine, Department of Internal Medicine, University Paris Diderot; E. Easley, MPH, Department of Family Medicine and Community Health, University of Pennsylvania; M. Gurcan, Medical student, Istanbul University Cerrahpasa Faculty of Medicine; T. Davis, BA, Department of Family Medicine and Community Health, University of Pennsylvania; A. Gul, MD, Professor of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Istanbul University Istanbul Faculty of Medicine; Y. Yazici, MD, Assistant Professor of Medicine, New York University School of Medicine, NYU Hospital for Joint Diseases; K. Zottenberg, patient-partner; S.N. Esatoglu, MD, Fellow in Rheumatology, Division of Rheumatology, Istanbul University Cerrahpasa Faculty of Medicine; B. Erer, MD, Associate Professor of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Istanbul University Istanbul Faculty of Medicine; S. Kamali, MD, Professor of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Istanbul University Faculty of Medicine; H. Yazici, MD, Professor of Rheumatology, Academic Hospital; P.F. Cronholm, MD, Associate Professor of Family Medicine, Department of Family Medicine and Community Health, University of Pennsylvania; P.A. Merkel, MD, MPH, Professor of Medicine and Epidemiology, Division of Rheumatology and Department of Biostatistics and Epidemiology, University of Pennsylvania. Address correspondence to Dr. P.A. Merkel, Division of Rheumatology, University of Pennsylvania, White Building, 5th Floor, 3400 Spruce St., Philadelphia, Pennsylvania 19104, USA. E-mail: Accepted for publication February 7, 2017.
    Objective: The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group has been working toward developing a data-driven core set of outcome measures for use in clinical trials of Behçet's syndrome [Behçet disease (BD)]. This paper summarizes the group's work through OMERACT 2016, discussions during the meeting, and the future research agenda.

    Methods: Qualitative patient interviews were conducted among 20 patients with BD who have different types of organ involvement. Read More

    Neuroimmunological response in neuro-Behçet's.
    Reumatol Clin 2017 Mar 27. Epub 2017 Mar 27.
    Laboratorio Central del Líquido Cefalorraquídeo (LABCEL), Facultad de Ciencias Médicas Miguel Enríquez, Universidad de Ciencias Médicas de La Habana, La Habana, Cuba.
    Behçet's disease is a chronic, multisystem, autoimmune vasculitis characterized by a triad of key signs. We report the case of a 16-year-old Caucasian girl, who complained of headache, dizziness and paresis of the left side of her body. After physical examination and ancillary tests, she was diagnosed with Behçet's disease with neurological manifestations (neuro-Behçet's disease). Read More

    Severe Cutaneous Adverse Drug Reactions in Pediatric Patients: A Multicenter Study.
    J Allergy Clin Immunol Pract 2017 May - Jun;5(3):757-763. Epub 2017 Mar 27.
    Division of Pediatric Allergy and Immunology, Department of Children's Health and Diseases, Faculty of Medicine, Mugla Sitki Kocman University, Mugla, Turkey. Electronic address:
    Background: The severe cutaneous adverse drug reactions (SCARs) are rare but could be life-threatening. These include drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome, toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis.

    Objective: The purpose of this study was the evaluation of the clinical characteristics of patients with the diagnosis of SCARs. Read More

    Successful treatment of Behçet's uveitis with Tocilizumab.
    Saudi J Ophthalmol 2017 Jan-Mar;31(1):42-44. Epub 2016 Dec 20.
    Prince Sultan Military Medical City, Department of Medicine, Rheumatology Division, Department of Ophthalmology, Saudi Arabia.
    A 33-year-old man with history of lymphoma was misdiagnosed as multiple sclerosis (MS) 18 months previously, developed intermediate uveitis, unresponsive to interferon, and corticosteroids. A diagnosis of Behçet's disease (BD) was made on the basis of recurrent orogenital ulceration, erythema nodosum, and a positive pathergy test. The patient was started on Tocilizumab and experienced an improvement in visual acuity and intraocular inflammation over the following 2 weeks. Read More

    Apremilast for treatment of recurrent erythema multiforme.
    Dermatol Online J 2017 Jan 15;23(1). Epub 2017 Jan 15.
    New York University, New York, NY.
    Recurrent erythema multiforme with oralinvolvement is therapeutically challenging.Apremilast has been used with success in resolvingthe oral aphthae of Behçet disease, prompting theuse of the drug in patients with oral erosions fromerythema multiforme. Three patients with oralerythema multiforme were given apremilast at dosesof 30-60mg daily. Read More

    The First Use of Pralidoxime in a Child With Rivastigmine Poisoning.
    Pediatr Emerg Care 2017 Mar 21. Epub 2017 Mar 21.
    From the Divisions of *Emergency Medicine and †Critical Care, Department of Pediatrics, Ege University School of Medicine; and ‡Critical Care Units, Dr Behçet Uz Children's Hospital, Izmir, Turkey.
    The aim of this report is to describe the successful use of pralidoxime in a pediatric patient who accidentally ingested 12 mg of rivastigmine and presented to the emergency department with weakness, drowsiness, hyporeactivity to environmental stimuli, and full cholinergic syndrome.

    Case: The patient presented to the emergency department 2 hours after a suspected ingestion of rivastigmine. He was sleepy but oriented and cooperative, hypotonic, and hyporeflexic and has a Glasgow Coma Scale score of 13 (E3M6V4). Read More

    Salivary Distinctiveness and Modifications in Males with Diabetes and Behçet's Disease.
    Biochem Res Int 2017 21;2017:9596202. Epub 2017 Feb 21.
    Department of Oral Medicine, Faculty of Dentistry, University of Damascus, Damascus, Syria.
    Oral diseases associated with systematic diseases as metabolic and vasculitic have been included in this paper. This will enhance our understanding of the salivary function in promoting healthy oral condition. The study investigates the effects of type I and type II diabetes mellitus in well-controlled diabetic patients, in addition to Behçet disease (BD) on saliva flow rate (SFR), pH, the decay, missing, and filled tooth (DMFT) index, glucose, and major earth-alkaline ions (Ca(2+) and Mg(2+)) compared to healthy males and age-matched controls. Read More

    Characteristics of Macular Edema in Behcet Disease after Intravitreal Bevacizumab Injection.
    J Ophthalmic Vis Res 2017 Jan-Mar;12(1):44-52
    Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
    Purpose: To investigate the effect of intravitreal bevacizumab (IVB) injection on macular edema (ME) secondary to Behcet's disease.

    Methods: This prospective case series included 15 patients with bilateral ME due to Behcet's disease. Intravitreal bevacizumab was injected into the more severely involved eye; the contralateral eye was evaluated as the control. Read More

    The utility of apoptosis inhibitor of macrophages as a possible diagnostic marker in patients with Crohn's disease.
    BMC Gastroenterol 2017 Mar 11;17(1):40. Epub 2017 Mar 11.
    Digestive and Lifestyle Diseases, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1, Sakuragaoka, Kagoshima, 890-8520, Japan.
    Background: Apoptosis inhibitor of macrophages (AIM) was initially identified as an apoptosis inhibitor that supports the survival of macrophages against various apoptosis-inducing stimuli, and AIM produced by macrophages may contribute to the pathogenesis of inflammatory bowel diseases (IBDs). However, there have been no reports on the kinetics of AIM in IBD and the impact of AIM on the pathogenesis of IBD. In this study, we aimed to investigate the diagnostic utility of levels of AIM and their correlation with the activity of Crohn's disease (CD) and IBD. Read More

    QUANTITATIVE ANALYSIS OF STRUCTURAL ALTERATIONS IN THE CHOROID OF PATIENTS WITH ACTIVE BEHÇET UVEITIS.
    Retina 2017 Mar 7. Epub 2017 Mar 7.
    *Department of Ophthalmology, School of Medicine, Koc University, Istanbul, Turkey; †Department of Ophthalmology, V.K. Foundation, American Hospital, Istanbul, Turkey; ‡Department of Ophthalmology, Koc University Hospital, Istanbul, Turkey; §Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey; ¶Department of Mathematics, Koc University, Istanbul, Turkey; **University of Lausanne, Lausanne, Switzerland; ††Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Lausanne, Switzerland; ‡‡Department of Family Medicine, School of Medicine, Marmara University, Istanbul, Turkey; and §§School of Medicine, Koc University, Istanbul, Turkey.
    Purpose: To quantitatively analyze in vivo morphology of subfoveal choroid during an acute attack of Behçet uveitis.

    Methods: In this prospective study, 28 patients with Behçet uveitis of ≤4-year duration, and 28 control subjects underwent enhanced depth imaging optical coherence tomography. A novel custom software was used to calculate choroidal stroma-to-choroidal vessel lumen ratio. Read More

    Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.
    Pediatr Cardiol 2017 Mar 7. Epub 2017 Mar 7.
    Department of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, 1374 St. No: 11 Alsancak, Izmir, Turkey.
    Both an excess of iron and iron deficiency (ID) may lead to significant cardiac problems. Parameters that represent ventricular repolarization heterogeneity, like QT dispersion (QTd), corrected QT dispersion (QTcd), the interval between the peak and the end of the T wave (Tp-e), and Tp-e dispersion, have not been evaluated in otherwise healthy children with low iron levels before. Here we assessed the effects of low iron storage on P wave dispersion (PWd), QTd, Tp-e intervals, and Tp-e dispersion in otherwise healthy children. Read More

    Increased Serum Antibody Titer against HPV-16 Antigen in Patients with Behçet's Disease.
    J Korean Med Sci 2017 Apr;32(4):599-604
    Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
    Quadrivalent human papillomavirus (HPV) vaccine has been reported to be significantly associated with Behçet's disease (BD). However, no reports have described HPV infection as a possible cause for the development of BD. The objective of this study was to evaluate whether anti-HPV immunoglobulin G (IgG) antibody titer is increased in BD. Read More

    Enhanced depth imaging optical coherence tomography in patients with different phases of Behcet's panuveitis.
    Can J Ophthalmol 2017 Feb 18;52(1):48-53. Epub 2016 Nov 18.
    Department of Ophthalmology, Baskent University, Faculty of Medicine, Ankara, Turkey.
    Objective: To evaluate the changes in choroidal thickness (CT) at 13 different points between "active," "remission," and "end-stage" phase of Behcet's panuveitis and compare this with the age, sex, and spherical equivalent matched healthy controls using enhanced depth imaging optical coherence tomography.

    Design: Prospective study.

    Participants: Eighty-five eyes of 45 patients with Behcet's panuveitis (19 eyes with active phase, 48 eyes with remission phase, and 18 eyes with end-stage phase) and 84 eyes of 42 controls were enrolled in this study. Read More

    Valved Conduit Attached to Left Ventricular Outflow Tract for Valve Detachment in Behçet's Disease.
    Ann Thorac Surg 2017 Mar;103(3):e301-e303
    Department of Cardiac Surgery, Union Hospital, Fujian Medical University, Fuzhou, Fujian, China.
    Standard aortic valve replacement for aortic regurgitation caused by Behçet's disease is frequently complicated by postoperative recurrent prosthetic valve detachment. To prevent this, we have developed a modified Bentall procedure, in which the valved conduit is proximally attached to the left ventricular outflow tract instead of to the fragile annulus, based on the fact that the left ventricular outflow tract myocardium is rarely involved in Behçet's disease. This modified Bentall procedure was performed in 5 Behçet's disease patients with prosthetic valve detachment after primary aortic valve replacement, and satisfactory results were achieved. Read More

    Association of sleep quality in Behcet disease with disease activity, depression, and quality of life in Korean population.
    Korean J Intern Med 2017 Mar 16;32(2):352-359. Epub 2017 Feb 16.
    Division of Rheumatology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea.
    Background/aims: Sleep disturbance is prime concern in patients with Behcet disease. The purpose of this study was to find out the effects of sleep quality, in Korean patients suffering from Behcet disease. We further investigated the relationship between depression, quality of life and the clinical findings of Behcet disease. Read More

    Circulating NK cells and their subsets in Behçet's disease.
    Clin Exp Immunol 2017 May 13;188(2):311-322. Epub 2017 Mar 13.
    Centre for Clinical and Diagnostic Oral Sciences, Institute of Dentistry, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
    Behçet's disease (BD) is an autoinflammatory, chronic relapsing/remitting disease of unknown aetiology with both innate and acquired immune cells implicated in disease pathogenesis. Peripheral blood natural killer (NK) cells and their CD56(Dim) /CD56(Bright) subsets were surface phenotyped using CD27 and CD16 surface markers in 60 BD patients compared to 60 healthy controls (HCs). Functional potential was assessed by production of interferon (IFN)-γ, granzyme B, perforin and the expression of degranulation marker CD107a. Read More

    HAX1 mutation positive children presenting with haemophagocytic lymphohistiocytosis.
    Br J Haematol 2017 May 7;177(4):597-600. Epub 2017 Feb 7.
    Department of Paediatric Haematology-Oncology, Dr. Behçet Uz Children Research and Training Hospital, Izmir, Turkey.
    The genetic basis of haemophagocytic lymphohistiocytosis (HLH) has not been elucidated in 10% of affected patients. In this study, we report four HLH episodes in three patients with HAX1 gene mutations. We screened the mutations associated with congenital neutropenia (CN) because the neutropenia persisted following HLH treatment. Read More

    [The value of different antibodies detection in diagnosis of rheumatism with uveitis].
    Zhonghua Yi Xue Za Zhi 2017 Jan;97(4):285-290
    Department of Clinical Laboratory, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
    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. Read More

    Behcet's disease.
    Clin Med (Lond) 2017 Feb;17(1):71-77
    University of Liverpool and director, National Behcet's Syndrome Centre of Excellence, Aintree University Hospital, Liverpool, UK.
    Behçet's disease (BD) is a chronic relapsing and remitting vasculitis of unknown aetiology. It has the capacity to affect almost all organ systems because of its potential to involve both arteries and veins of all sizes, resulting in significant organ-threatening morbidity and mortality. Traditionally known as the 'silk road' disease, it has a worldwide occurrence. Read More

    Multiple Aneurysms and a Transplanted Kidney in Behçet Disease.
    Vasc Endovascular Surg 2017 Feb 1;51(2):108-110. Epub 2017 Feb 1.
    1 Department of Vascular Surgery, University Hospital Birmingham, Birmingham, United Kingdom.
    Arterial manifestation of Behçet disease represents a challenging clinical scenario with a potential for fatal complications. This case depicts the surgical management of a 4.5-cm infrarenal aortic aneurysm and a 6-cm left renal artery aneurysm in a patient with known Behçet disease. Read More

    Treating an Adolescent with Long QT Syndrome for Bipolar Disorder: A Case Presentation.
    Psychopharmacol Bull 2017 Jan;47(1):33-39
    Drs. Önen, Kutlu, Erkuran, MD, Dr. Behcet Uz Children's Hospital Department of Child and Adolescent Psychiatry, Izmir, Turkey.
    Objectives: Long QT syndrome (LQTS) is described as the development of sudden syncope attacks or death as a result of ventricular tachycardia (VT) episodes that might be observed as elongated QT interval in electrocardiography (ECG). Implantable Cardioverter Defibrillator (ICD) is recommended as first-line treatment for the condition in guidelines. We aimed to present an adolescent recently diagnosed with Bipolar Disorder (BD) who had LQTS that was treated with ICD, discussing her follow up and treatment along with relevant literature. Read More

    Panniculitis: A summary.
    Semin Diagn Pathol 2017 May 27;34(3):261-272. Epub 2016 Dec 27.
    Section of Dermatopathology,Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, USA. Electronic address:
    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. Read More

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