Pediatric Behçet's Disease.

Behçet's Disease (BD) is a systemic vasculitis firstly described as a disorder causing aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely unique distribution characterized by the highest incidence in communities living along the historical Silk road. Although our understanding of the etiopathogenesis of BD has expanded over time, there are still lots of unidentified points in the underlying mechanisms of the disease.

Macroscopic T-wave alternans in a patient presenting with hepatic encephalopathy.

Macroscopic T-wave alternans (TWA) is the beat-to-beat variation in the amplitude or shape of the T wave on a surface electrocardiogram (ECG) and known to be a harbinger of impending malignant ventricular arrhythmias such as polymorphic ventricular tachycardia. We herein report a case with hepatic encephalopathy, who developed TWA, followed by polymorphic ventricular tachycardia.

Characteristics and outcomes of COVID-19 inpatients who underwent psychiatric consultations.

Patients hospitalized with COVID-19 are at risk of developing many neuropsychiatric disorders, due to the effects of the disease on the brain and the psychosocial pressures of having the disease. The aim of the present study was to evaluate the characteristics and outcomes of patients who were hospitalized with a diagnosis of COVID-19, who underwent psychiatric consultations. The medical records of 892 patients hospitalized due to COVID-19 and the 89 among them who requested psychiatric consultations were analyzed retrospectively.

Sarcopenia quality-of-life questionnaire (SarQoL)®: translation, cross-cultural adaptation and validation in Turkish.

Background: The sarcopenia quality-of-life (SarQoL)® questionnaire is a multidimensional sarcopenia specific tool designed for community dwelling older adults.

Aims: The aim of this study was to translate, to cross-culturally adapt and validate the SarQoL® questionnaire to assess sarcopenia-related quality of life in Turkish older adults.

Methods: The validation process was performed in two sections: the first section constituted the translation with cross-cultural adaptation of SarQoL® into Turkish.

Influenza and pneumococcus vaccination rates in pediatric dialysis patients in Europe: recommendations vs reality A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group study.

Background/aim: Children on dialysis are under increased risk of influenza and invasive pneumococcal disease. Although vaccination against these microorganisms are recommended in dialysis patients and despite the fact that these vaccines can reduce disease burden and rates of hospitalization due to infection, vaccination rates are below expected and desired. We aimed to evaluate influenza and pneumococcal vaccination and infection rates in European pediatric dialysis centers.

The role of asymptomatic and pre-symptomatic infection in SARS-CoV-2 transmission-a living systematic review.

Background: Reports suggest that asymptomatic individuals (those with no symptoms at all throughout infection) with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are infectious, but the extent of transmission based on symptom status requires further study.

Purpose: This living review aims to critically appraise available data about secondary attack rates from people with asymptomatic, pre-symptomatic and symptomatic SARS-CoV-2 infection.

Data Sources: Medline, EMBASE, China Academic Journals full-text database (CNKI), and pre-print servers were searched from 30 December 2019 to 3 July 2020 using relevant MESH terms.

Cross-cultural adaptation of the Fresno Test for Turkish language.

Objective: National and international medical organizations and boards have recognized the importance of Evidence Based Medicine (EBM) and emphasized that EBM training should be included in medical education programs. Although some Turkish medical schools have developed and implemented EBM training programs, no validated Turkish language assessment tool has been available to compare the effectiveness of these training programs to national or international standards. The aim of this study is to cross-culturally adapt the Fresno Test, which is a validated English language tool utilized worldwide in the assessment of EBM training.

Immunogenicity of Infliximab Among Patients With Behçet Syndrome: A Controlled Study.

Background: Immunogenicity of tumor necrosis factor alpha inhibitors (TNFis) has been recognized as an important problem that may cause loss of efficacy and adverse events such as infusion reactions. TNFis are being increasingly used among patients with Behçet syndrome (BS) and scarce data exist on this topic.

Objective: We aimed to investigate the prevalence of anti-infliximab (IFX) antibodies in patients with Behçet syndrome together with suitable controls.

Anti-nuclear antibody testing in children: How much is really necessary?

Background: Anti-nuclear antibody (ANA) testing is most commonly ordered by general pediatricians to evaluate children with musculoskeletal system complaints. Given the limited utility of the test, we aimed to estimate the effectiveness of ordering ANA testing in childhood.

Methods: Children referred to our department to be examined due to positive ANA findings between 2008 and 2020 were included in the study.

Childhood-onset versus adult-onset Takayasu arteritis: A study of 141 patients from Turkey.

Aim: To compare childhood-onset (c-TAK) versus adult-onset Takayasu arteritis (a-TAK) patients for vascular involvement, disease activity, damage, and treatment.

Methods: Patient charts from two tertiary-care centers of a pediatric and adult rheumatology clinic were reviewed. Adult patients diagnosed before the age of 18 years were included in the childhood-onset group.

Systolic and Diastolic Cardiac Functions in Juvenile Spondyloarthropathies.

Background/objective: Juvenile spondyloarthropathies (JSpAs) are a group of inflammatory diseases characterized by asymmetric peripheral arthritis (especially in lower extremities), axial skeleton involvement, and enthesitis. Although cardiovascular findings of inflammatory diseases such as juvenile systemic lupus erythematosus (SLE) and juvenile scleroderma (SD) are well documented, there are only a few studies assessing the cardiovascular consequences of JSpA in the literature.

Methods: Forty patients with JSpA and 20 healthy controls were included into this cross-sectional study.

Local aromatase activity alterations in breast cancer tissues: A potential way of decision support for clinicians.

Background And Aims: It is becoming evident that local estrogen exposure is important in postmenopausal breast cancer patients. The microenvironment is established by breast stromal cells based on communication with tumor cells that is essential to cancer development, invasion, and metastasis. Here we investigated aromatase activity levels in both tumor and matched stromal tissues by showing their impact on the manufacturing of local estrogen and tumor progression in cases of invasive ductal carcinoma (IDC).

Consensus-based recommendations for the management of juvenile systemic sclerosis.

Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no international consensus exists with regard to its assessment and treatment. This SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based on expert opinion informed by the best available evidence, provides recommendations for the assessment and treatment of patients with JSSc with a view to improving their outcome. Experts focused attention not only on the skin assessment but also on the early signs of internal organ involvement whose proper treatment can significantly affect the long-term outcome.

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis.

Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc.

Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD.

Results: Eighty-six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison.

Tocilizumab therapy in juvenile systemic sclerosis: a retrospective single centre pilot study.

To evaluate the efficacy and safety of anti-interleukin (IL)-6 receptor antibody tocilizumab (TCZ) as a treatment option of juvenile systemic sclerosis (JSS). Nine JSS patients were assigned to a TCZ, additionally to conventional treatment (steroids, methotrexate, mycophenolate-mofetil). The modified Rodnan skin score (mRSS), carbon-monoxide diffusion capacity (DLCO), thorax high-resolution tomography (HRCT), patient global assessment (PGA) and Juvenile Systemic Sclerosis Severity (J4S) score were used to explore the efficacy of treatment.

G-protein Coupled Estrogen Receptor Expression in Growth Hormone Secreting and Non-Functioning Adenomas.

Purpose: To evaluate the expression of G-protein coupled estrogen receptor (GPER1), aromatase, estrogen receptor α (ERα), estrogen receptor β (ERβ), pituitary tumor transforming gene (PTTG), and fibroblast growth factor 2 (FGF2) in GH-secreting and non-functioning adenomas (NFA).

Methods: Thirty patients with acromegaly and 27 patients with NFA were included. Gene expression was determined via quantitative reverse transcription polymerase chain reaction (QRT-PCR).

Independent risk factors for resolution of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome within 4 years after the disease onset.

Background/objective: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a polygenic disease with unknown etiology. In this retrospective cohort study, we aimed to evaluate the risk factors for the resolution of PFAPA syndrome within 4 years after the onset.

Methods: In total, 466 patients with PFAPA syndrome that are being followed up our department were included into the study.

The role of Mediterranean fever gene variants in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

This study was conducted to investigate the relationship between clinic features and Mediterranean fever gene (MEFV) variants in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. In total, 167 patients with PFAPA syndrome were included in the study. Female:male ratio of the patients was 0.

Evaluation of the thyroid disorders in children with familial Mediterranean fever.

Although it is well-known that autoimmune thyroid diseases are more common in most of the autoimmune connective tissue diseases, the relationship between autoinflammatory diseases and autoimmune thyroid diseases has not well-evaluated yet and still remains unclear. The aim of this study was to investigate the frequency of autoimmune diseases of the thyroid gland and to evaluate thyroid function tests in children with familial Mediterranean fever. Thyroxine, thyroid-stimulating hormone, and thyroid autoimmune markers such as thyroid peroxidase and thyroglobulin antibodies, and thyroid ultrasound findings of 133 patients with familial Mediterranean fever and 70 healthy controls were evaluated.

Risk factors associated with adverse fetal outcomes in pregnancies affected by Coronavirus disease 2019 (COVID-19): a secondary analysis of the WAPM study on COVID-19.

Objectives To evaluate the strength of association between maternal and pregnancy characteristics and the risk of adverse perinatal outcomes in pregnancies with laboratory confirmed COVID-19. Methods Secondary analysis of a multinational, cohort study on all consecutive pregnant women with laboratory-confirmed COVID-19 from February 1, 2020 to April 30, 2020 from 73 centers from 22 different countries. A confirmed case of COVID-19 was defined as a positive result on real-time reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assay of nasal and pharyngeal swab specimens.