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Turk J Pediatr 2022 ;64(2):408-411

Department of Pediatric Infectious Diseases, University of Health Sciences Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, İzmir.

Background: As an extremely rare entity reported in children, Rasmussen`s aneurysm is an inflammatory pseudo-aneurysmal dilatation of a branch of the pulmonary artery adjacent to or within a tuberculous cavity.

Case: Here, we reported a 9-year-old child with Down syndrome who presented with massive hemoptysis. Endovascular coil embolization was performed for Rasmussen`s aneurysm. Read More

Turk J Pediatr 2022 ;64(2):364-374

Department of Pediatric Neurology, University of Health Sciences, Dr. Behçet Uz Children's Education and Research Hospital, İzmir.

Background: Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a very rare autosomal recessive disorder caused by mutations in the immunoglobulin μ-binding protein-2 (IGHMBP2) gene on chromosome 11q13.2-q13.4. Read More

Rev Med Interne 2022 May 20. Epub 2022 May 20.

Service de Cardiologie B de la maternité Souissi, CHU Ibn Sina, Université Mohammed V de Rabat, 10100 Rabat, Maroc; Université Mohamed V, Faculté de Médecine et Pharmacie, Rabat, Maroc.

Amino Acids 2022 May 23. Epub 2022 May 23.

Department of Biochemistry, Faculty of Medicine, Selcuk University, 42130, Konya, Turkey.

Behçet disease (BD) is an inflammatory, multisystemic vasculitis of unknown etiopathogenesis. However, innate and adaptive immune system involvement and immune-mediated networks play a vital role in the inflammatory cascade. Indoleamine 2,3-dioxygenase 1 (IDO1) is activated in chronic inflammatory states and catalyzes the first and rate-limiting step of tryptophan (TRP) metabolism along the kynurenine pathway (KP). Read More

Int J Ophthalmol 2022 18;15(5):813-819. Epub 2022 May 18.

Department of Biochemistry, Faculty of Medicine, Urmia University of Medical Sciences, Urmia 5756115111, Iran.

Aim: To investigate effects of anti-TNF biologic drugs on uveitis severity (comparing visual acuity logMAR levels) in Behçet patients.

Methods: Three databases PubMed, Scopus, and the Web of Science were searched for qualified papers focusing on the anti-TNF-α factors treatment in Behçet's disease (BD)-associated uveitis. Studies that were designed pre and post anti-TNF drug treatment, were selected. Read More

Curr Rheumatol Rev 2022 May 18. Epub 2022 May 18.

Department of neurology. Hospital Universitario de Torrejón, Spain.

Systemic inflammatory diseases could produce neurologic complications, and they are frequently incorporated in the differential diagnosis of neurological symptoms. There are well-established criteria to meet the diagnosis of neurologic manifestations of these systemic diseases. However, the range of clinical presentations varies in each condition, and the prevalence of these complications differs between studies. Read More

Ther Umsch 2022 Jun;79(5):269-276

Klinik für Rheumatologie, Department Innere Medizin, Kantonsspital St. Gallen.

Behçet's disease Behçet's disease (BD) represents a diagnostic and therapeutic challenge for the treating physician. The rarity of the disease and its heterogeneous clinical presentations hamper physicians in easily gaining knowledge about the disease. Moreover, the diagnosis of BD commonly relies on clinical grounds only and treatment decision are guided by relatively few data from clinical trials. Read More

Pract Neurol 2022 May 16. Epub 2022 May 16.

Department of Neurology, Imperial College Healthcare NHS Trust, London, UK.

Osmotic demyelination syndrome characteristically follows rapid correction of hyponatraemia. We present a young woman with a subacute progressive brainstem syndrome and diffuse pontine signal abnormality on MR imaging, diagnosed as osmotic demyelination syndrome. The case posed a diagnostic challenge due to comorbid Behçet's disease and the absence of significant fluctuation in her serum sodium concentration. Read More

Front Pediatr 2022 28;10:864609. Epub 2022 Apr 28.

Division of Neonatology, Department of Pediatrics, Faculty of Medicine, Konya Hospital, Başkent University, Konya, Turkey.

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion. Read More

Cureus 2022 Apr 12;14(4):e24064. Epub 2022 Apr 12.

Department of Internal Medicine, Dr. Ruth K. M. Pfau Civil Hospital Karachi, Karachi, PAK.

Behcet's disease (BD), also known as Behcet's syndrome, is a rare, chronic, autoimmune disorder of unknown origin. Its manifestations are thought to be caused by vasculitis, resulting in damage to blood vessels of all sizes throughout the body. We report a 25-year-old Pakistani male who is sexually active and presents with a one-year history of shortness of breath, cough, exertional dyspnea, and neck and facial swelling. Read More

Dermatol Ther 2022 May 15:e15585. Epub 2022 May 15.

Department of Dermatology, İstanbul Training and Research Hospital, University of Health Sciences, İstanbul, Turkey.

Behçet's disease (BD) is a chronic systemic vasculitis with a wide range of clinical findings. It has both autoinflammatory and autoimmune features and manifests with recurrent inflammatory attacks involving the innate immune system. Recently, autoinflammation has started to take place in the pathogenesis of intervertebral disc degeneration. Read More

Int Immunopharmacol 2022 May 10;109:108825. Epub 2022 May 10.

Department of Community Nutrition, Faculty of Nutrition and Food Sciences, Tabriz University of Medical Sciences, Tabriz, Iran. Electronic address:

Background: Overproduction of NLRP3 inflammasome complex is one of the causes of Behcet's disease's (BD) auto-inflammatory nature. The aim of current study was to examine the effect of zinc supplementation on NLRP3 inflammasome expression; as well as clinical manifestations of BD.

Methods: In this double-blind parallel placebo-controlled randomized clinical trial, 50 BD patients were randomly allocated into either zinc gluconate (30 mg/day elemental zinc) or placebo groups for 12 weeks. Read More

Arq Bras Oftalmol 2022 May 9. Epub 2022 May 9.

Department of Radiology, Faculty of Medicine, Adnan Menderes University, Aydin, Turkey.

Purpose: The study aimed to investigate the correlation between arterial hemodynamics measured by color Doppler ultrasonography and retinal microarchitecture parameters determined by spectral-domain optical coherence tomography (SD-OCT) in pseudoexfoliation glaucoma.

Methods: This prospective study included 82 participants. Peripapillary retinal nerve fiber layer, ganglion cell inner plexiform layer, and ganglion cell complex values were measured. Read More

RMD Open 2022 05;8(1)

Sorbonne University, AP-HP, Tenon Hospital, Internal Medicine Department, 4 rue de la Chine, 75020, Paris, France; national Reference center for autoinflammatory diseases and AA amyloidosis (CEREMAIA), Tenon Hospital, Paris, France

Objective: There is little known about SARS-CoV-2 infection in patients with systemic autoinflammatory disease (SAID). This study aimed to describe epidemiological features associated with severe disease form and death. Mortality between patients with and without SAID hospitalised for SARS-CoV-2 infection was compared. Read More

Sisli Etfal Hastan Tip Bul 2022 28;56(1):161-165. Epub 2022 Mar 28.

Department of Pediatric Hematology and Oncology, Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, Izmir, Turkey.

Autosomal recessive osteopetrosis is also known as infantile malignant osteopetrosis (IMO). The clinical course is often serious and if left untreated, it is fatal in the 1st year of life. Diagnosis is challenging and often delayed or misdiagnosed. Read More

World J Pediatr 2022 May 4. Epub 2022 May 4.

Department of Pediatric Infectious Diseases, University of Health Sciences Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital, İzmir, Turkey.

Background: There are limited numbers of studies focusing on renal effects of coronavirus disease 2019 (COVID-19) infection and proximal tubular dysfunction in children with COVID-19 infections. The purpose of this study was to evaluate the functions of the proximal tubule in hospitalized children with confirmed acute COVID-19.

Methods: The children who were hospitalized for confirmed COVID-19 were included in this prospective descriptive analysis. Read More

Clin Exp Hypertens 2022 Jul 3;44(5):480-486. Epub 2022 May 3.

Department of Internal Medicine, Bagcilar Medilife Hospital, Istanbul, Turkey.

Objectives: The incidence of cardiovascular disease is increased in patients with Behcet's disease (BD). Proprotein convertase subtilisin/kexin type 9 (PCSK9) causes the acceleration of atherosclerosis. We aimed to investigate whether there is a relationship between PCSK9 with carotid artery intima-media thickness (cIMT), a marker of subclinical atherosclerosis, and BD disease activity. Read More

Int J Pediatr Otorhinolaryngol 2022 Apr 25;158:111153. Epub 2022 Apr 25.

İzmir Katip Çelebi University, Faculty of Medicine, Department of Pediatric Rheumatology, İzmir, Turkey.

JAAD Int 2022 Jun 18;7:137-143. Epub 2022 Apr 18.

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas.

Background: Eponyms are ubiquitous in dermatology; however, their usage trends have not been studied.

Objective: To characterize the usage of eponyms in dermatology from 1880 to 2020.

Methods: Candidate eponyms were collected from a textbook and an online resource. Read More

Allergy 2022 May 1. Epub 2022 May 1.

Faculty of Medicine, Pediatric Allergy and Immunology, Necmettin Erbakan University, Konya, Turkey.

Background: Lipopolysaccharide-responsive beige-like anchor protein (LRBA) deficiency and cytotoxic T-lymphocyte protein-4 (CTLA-4) insufficiency are recently described disorders that present with susceptibility to infections, autoimmunity, and lymphoproliferation. Clinical and immunological comparisons of the diseases with long-term follow-up have not been previously reported. We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting the genetic defect in patients with LRBA deficiency and CTLA-4 insufficiency. Read More

Arthritis Res Ther 2022 04 29;24(1):98. Epub 2022 Apr 29.

Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan'an West Road, Shanghai, 200040, People's Republic of China.

Background: Behçet's syndrome (BS) is a rare variant vasculitis which can involve the eyes and gastrointestinal systems. However, ocular involvement rarely overlaps with intestinal lesions. This study aimed to compare the clinical characteristics and laboratory parameters of ocular BS and intestinal BS patients in China and analyze the differences between two key phenotypes to verify the heterogeneous conditions in BS patients. Read More

Semin Arthritis Rheum 2022 Apr 15;55:152010. Epub 2022 Apr 15.

Harvard Medical School, Massachusetts General Hospital, Boston, MA, United States.

Glucocorticoids (GCs) have been the cornerstone of treating dozens of inflammatory conditions for more than seven decades. GC toxicity is ubiquitous in both clinical trials and clinical practice, and toxicities associated with GC use are central to the experience of most patients being treated for immune-mediated conditions. These conditions span the full range of medical specialties, including rheumatology, nephrology, gastroenterology, neurology, pulmonology, ophthalmology, and others. Read More

Clin Exp Rheumatol 2022 May 29;40(4):863-864. Epub 2022 Apr 29.

Department of Experimental and Clinical Medicine, University of Firenze, and SOD Interdisciplinary Internal Medicine, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Italy.

Turk J Ophthalmol 2022 04;52(2):142-146

Gazi University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey

Corticosteroid-induced central serous chorioretinopathy (CSCR) has been reported to develop in many intraocular inflammatory diseases and usually resolves spontaneously after discontinuation of corticosteroids. Patients without any improvement may require alternative therapies. In this case report, we present the case of a 35-year-old man with Behçet's disease who had complaints of decreased vision due to CSCR in his left eye while using systemic corticosteroids along with cyclosporine and azathioprine. Read More

Medicine (Baltimore) 2022 Apr 15;101(15):e29189. Epub 2022 Apr 15.

Department of Ophthalmology, National Cheng Kung University Hospital, Tainan, Taiwan.

Rationale: Although single-cytokine inhibitors can be considered in treating severe or refractory Behçet disease (BD), these biologic agents are associated with potential therapeutic failure due to the multi-cytokine pathogenesis involving Th1- and Th17-type cytokines with activated Janus kinase/signal transducer and activator of transcription signaling pathways. Notably, there is an increasing trend toward the use of small-molecule targeted drug tofacitinib (TOF), a pan-Janus kinase inhibitor, with immediate-release formulations for treating patients with severe or refractory systemic vasculitis involving different vessel sizes. Despite no reported efficacy of extended-release formulations in refractory BD yet, such a dosage form has pharmacokinetic parameters that are comparable to those of conventional immediate-release formulations. Read More

Clin Case Rep 2022 Apr 20;10(4):e05626. Epub 2022 Apr 20.

Department of Neurology Neurosciences Institute Hamad Medical Corporation Doha Qatar.

Paroxysmal dysarthria and ataxia (PDA) is a rare neurological manifestation of stereotyped attacks of sudden ataxic symptoms lasts for few seconds to minutes. We report a case of PDA in a 61-year-old male with a solitary homogenously enhancing solitary midbrain lesion and positive HLA-B51 (Allele 2), controlled with lacosamide. Read More

Am J Med Sci 2022 Apr 22. Epub 2022 Apr 22.

Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Cairo University, Cairo, Egypt.

Background: Interleukin 37 (IL-37) is an anti-inflammatory cytokine previously studied in Behçet's disease (BD) and atherosclerosis. However, little is known about its relation to macro and microcirculations in BD. Previous studies relied mainly on common carotid artery (CCA) intima-media thickness (IMT) and ankle brachial index (ABI) to study atherosclerosis in BD with conflicting results. Read More

J Mol Neurosci 2022 Apr 24. Epub 2022 Apr 24.

Department of Pediatric Neurology, University of Health Sciences, Dr. Behcet Uz Children's Training and Research Hospital, Izmir, Turkey.

The term "epileptic encephalopathy" is used to describe a possible relationship between epilepsy and developmental delay. The pathogenesis of developmental encephalopathies, independent of epilepsy, can be defined by genetic control mechanisms. The aim of this study was to investigate the use of miRNAs as serum biomarkers for the determination and discrimination of epileptic encephalopathies. Read More

Cells 2022 Apr 12;11(8). Epub 2022 Apr 12.

Laboratory of Transmission, Control and Immunobiology of Infections, Institut Pasteur de Tunis, Tunis 1002, Tunisia.

Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Read More

J Mycol Med 2022 Apr 6;32(3):101277. Epub 2022 Apr 6.

Division of Pediatric Intensive Care Unit, Dr. Behcet Uz Children's Disease and Pediatric Surgery Training and Research Hospital, Izmir, Turkey.

Objective: Candida species are among the most prevalent microorganisms in pediatric critical care units that cause central line-associated bloodstream infections. The goal of this study was to assess the therapeutic benefit of central line bundle for the prevention of Candida species-related bloodstream infections in pediatric intensive care units.

Design: The study covered the period from January 1, 2009, to December 31, 2019. Read More