Association of IL-23R and IL-10 variations with Behçet disease: a genetic analysis study.

Behçet disease (BD) is an autoimmune and autoinflammatory disease mainly affecting the Silk Road countries. The interindividual severity of BD depends on differences in the polymorphic profiles of the patients. One of the most prominent markers, HLA-B51 positivity, is also observed in 40-60% of patients with BD on the Silk Road.

The IL-25/ILC2 axis promotes lung cancer with a concomitant accumulation of immune-suppressive cells in tumors in humans and mice.

Background: Group 2 innate lymphoid cells (ILC2) can be activated by interleukin (IL)-33 or IL-25. IL-25-activated ILC2 cells help protect the host against helminth infection while exacerbating allergic-like inflammation and tissue damage in the lung. In the context of cancer, IL-33-activated ILC2 cells were found to bear anti-tumoral functions in lung cancer while IL-25-activated ILC2 cells promoted tumorigenesis in colorectal cancer.

Exploration of Flexible Pes Planus as a Potential Risk Factor for Cardiac Pathologies in Pediatric Cases.

Objective: The aim of this study is to investigate the relationship between flexible pes planus (FPP) and cardiac pathologies in pediatric patients, with a particular emphasis on hypermobility status.

Methods: Between January and June 2022, a retrospective screening was conducted on a total of 68 patients aged between 6 and 18 years who had been diagnosed with FPP. Eight patients were excluded from the study due to suspicion of connective tissue or neuromuscular diseases following systemic examinations conducted by a pediatric specialist.

Gray zone in the spectrum of autoinflammatory diseases: familial Mediterranean fever accompanying periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: single-center experience.

Despite the advanced knowledge concerning autoinflammatory diseases (AID), more data regarding the optimal treatment options and outcomes of the children who met the criteria of more than one AID are required. This study aimed to describe the demographic and clinical characteristics of children from familial Mediterranean fever (FMF)-endemic countries who meet both the FMF and the periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome criteria. Moreover, we aimed to measure the response rates to colchicine and tonsillectomy and evaluate the factors affecting the colchicine response in these patients.

Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis.

Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.

Hypothalamic-Pituitary Axis Function and Adrenal Insufficiency in COVID-19 Patients.

The outbreak of COVID-19 has affected more than half a billion people worldwide and caused more than 6 million deaths since 2019. The responsible virus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), primarily affects the lungs, but it has multisystemic effects. It is well known that dysfunction of multiple endocrine organs may occur during or after COVID-19.

The impact of COVID-19 on clinical course and treatment among patients with juvenile systemic sclerosis.

Objectives: This study aimed to explore the influence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic among patients with juvenile systemic sclerosis (JSS).

Patients And Methods: Twenty-seven patients (22 females, 5 males; mean age: 20 years; range, 17 to 22 years) diagnosed with JSS and followed up at the department of pediatric rheumatology were included in the cross-sectional study. A web-based survey was performed by focusing on patients' complaints, accessibility to health care, and compliance with routine treatment from January 1, 2021, to January 10, 2021.

Uniportal pure robotic-assisted thoracic surgery-technical aspects, tips and tricks.

The uniportal access for robotic thoracic surgery presents itself as a natural evolution of minimally invasive thoracic surgery (MITS). It was developed by surgeons who pioneered the uniportal video-assisted thoracic surgery (U-VATS) in all its aspects following the same principles of a single incision by using robotic technology. The robotic surgery was initially started as a hybrid procedure with the use of thoracoscopic staplers by the assistant.

Malnutrition risk in hospitalized patients measured with Nutrition Risk Screening 2002 tool and its association with in-hospital mortality.

Objective: Malnutrition is related to increased morbidity, mortality, and costs. NRS-2002 is a practical malnutrition risk (MR) screening tool approved by the European Society for Clinical Nutrition and Metabolism (ESPEN) for inpatients. We aimed to reveal the inpatient MR using NRS-2002, and to examine the relationship between MR and in-hospital mortality.

A patient-driven registry on Behçet's disease: the AIDA for patients pilot project.

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD).

Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry.

Proposed Response Parameters for Twelve-Month Drug Trial in Juvenile Systemic Sclerosis: Results of the Hamburg International Consensus Meetings.

Objective: Juvenile systemic sclerosis (SSc) is an orphan disease, associated with high morbidity and mortality. New treatment strategies are much needed, but clearly defining appropriate outcomes is necessary if successful therapies are to be developed. Our objective here was to propose such outcomes.

Radiation recall myositis with pazopanib in a patient with soft tissue sarcoma.

Introduction: Radiation recall reaction is an acute inflammatory reaction confined to previously irradiated areas that are most commonly triggered by the administration of anti-cancer agents after radiotherapy. Radiation recall myositis is a relatively rare form of radiation recall reaction.

Case Report: Here we report a 29-year-old female patient who suffered from metastatic monophasic synovial sarcoma.

The relations of circulating agouti-related peptide and leptin with altered sleep architecture in patients with active Cushing's disease: a pilot study.

Aim: To evaluate sleep architecture of patients with Cushing's disease (CD) and to explore whether agouti-related peptide (AgRP) and/or leptin play a permissive role in sleep alterations in patients with active CD.

Methods: We performed polysomnography on 26 patients with active CD and age 26 age- and sex-matched control subjects. Blood samples were obtained from all participants for the analyzes of AgRP and leptin.

Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort.

Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.

Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months.

Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males.

The efficacy of a single-dose anakinra injection during disease attack in pediatric familial Mediterranean fever.

The aim of this retrospective study is to evaluate the efficacy of a single-dose anakinra during familial Mediterranean fever (FMF) attacks and its effect on the duration, severity, and frequency of attacks. The patients with FMF who had disease episode and received a single-dose anakinra during disease episode between December 2020 and May 2022 were included. Demographic characteristics, MEFV gene variants detected, concomitant medical conditions, demographics of recent and previous episodes, laboratory findings and length of hospital stay were recorded.

Separation and quantification of the urinary enantiomers of 2-hydroxyglutaric acid by capillary electrophoresis with capacitively coupled contactless conductivity detection: Application to the diagnosis of D- and L-2-hydroxyglutaric aciduria.

2-hydroxyglutaric aciduria is an inherited neurometabolic disorder with two major types: D-2-hydroxyglutaric aciduria and L-2-hydroxyglutaric aciduria. An easy and fast capillary electrophoresis system combined with a capacitively coupled contactless conductivity detection method was developed for the enantioseparation and determination of D- and L-2-hydroxyglutaric acid in urine. D- and L-2-hydroxyglutaric acids were separated using vancomycin as the chiral selector.

Therapeutic approach to central nervous system involvement of Behçet's disease.

Background: Neurologic involvement in Behçet's disease (BD) represents a major cause of disease morbidity and mortality. Early recognition and timely treatment represent crucial aspects that aim at preventing long-term disability. The absence of robust and evidence-based studies further complicates the management of neuro-BD (NBD).

World federation of interventional and therapeutic neuroradiology (WFITN) federation assembly neurointerventional surgery safety checklist.

Over the last 10 years, there has been a rise in neurointerventional case complexity, device variety and physician distractions. Even among experienced physicians, this trend challenges our memory and concentration, making it more difficult to remember safety principles and their implications. Checklists are regarded by some as a redundant exercise that wastes time, or as an attack on physician autonomy.

The First Case Report of 47,XXY/46,XX/46,XY Mosaic Klinefelter Syndrome Patient With Mixed Connective Tissue Disorder.

Klinefelter syndrome (KS) mosaicism 47,XXY/46,XX/46,XY is an extremely rare disorder. Mixed connective tissue disorder (MCTD) is a systemic rheumatological disease with overlapping characteristic features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM)/dermatomyositis (DM), and rheumatoid arthritis (RA). It contains a higher titer level of U1-RNP and anti-RNP antibodies.

Altered thalamic volume in patients with mild autonomous cortisol secretion: a structural brain MRI study.

Purpose: To compare thalamic volume and cognitive functions of patients with mild autonomous cortisol secretion (MACS) with control subjects and patients with overt Cushing's syndrome (CS).

Methods: In this cross-sectional study, volumes of regions of interest were assessed using 3 T magnetic resonance imaging and a voxel-based morphometry approach in 23 patients with MACS, 21 patients with active CS, 27 patients with CS in remission, and 21 control subjects. Cognitive functions were assessed using validated questionnaires.

Cortices of Fibula and Tibia Can Provide Landmarks for Accurate Syndesmosis Fixation Angle: Computed Tomography Validation of Angle Bisector Method.

Anatomic syndesmosis reduction is necessary to restore ankle biomechanics and prevent poor clinical outcomes, but malreduction can be encountered frequently since the ideal fixation angle varies between patients and fixation levels. This study aimed to validate the angle bisector method to reveal whether it provides an accurate syndesmotic fixation angle that is patient- and level-specific. Lower extremity CT angiography of 50 consecutive patients (25 male, 25 female) without evident ankle pathology were evaluated.

Comparison of eversion carotid endarterectomy under local anesthesia and eversion/conventional carotid endarterectomy under general anesthesia.

Introduction: Studies searching outcomes of eversion carotid endarterectomy (E-CEA) under local anesthesia are lacking.

Aim: To evaluate the postoperative outcomes of E-CEA under local anesthesia and compare it with E-CEA/Conventional CEA under general anesthesia in symptomatic or asymptomatic patients.

Material And Methods: From February 2010 to November 2018 a total of 182 patients (143 males, 39 females; mean age: 69.

Comparison of uniportal robotic-assisted thoracic surgery pulmonary anatomic resections with multiport robotic-assisted thoracic surgery: a multicenter study of the European experience.

Background: Robotic-assisted thoracic surgery (RATS) has seen increasing interest in the last few years, with most procedures primarily being performed in the conventional multiport manner. Our team has developed a new approach that has the potential to convert surgeons from uniportal video-assisted thoracic surgery (VATS) or open surgery to robotic-assisted surgery, uniportal-RATS (U-RATS). We aimed to evaluate the outcomes of one single incision, uniportal robotic-assisted thoracic surgery (U-RATS) against standard multiport RATS (M-RATS) with regards to safety, feasibility, surgical technique, immediate oncological result, postoperative recovery, and 30-day follow-up morbidity and mortality.