Search

OBJECTIVE: Discrete supravalvular aortic stenosis (SAS) is known to involve the whole aortic root. Some surgeons have therefore changed their approach from relief of obstruction using a single-patch to symmetric reconstruction of the whole aortic root - three-patch technique. The advantages are said to be preserved long-term aortic valve function and allowance for growth. This is unproven. We compare growth and aortic root geometry in patients who have undergone relief of discrete SAS using either single-or three-patch technique. METHODS: Twenty-five patients (14 male, 11 female, mean age of 11+/-4 years, range 4-18) underwent surgery for discrete SAS. No patients with diffuse SAS were included in this retrospective analysis. Twelve patients had features of Williams syndrome. Five patients had other concomitant procedures. A single-patch was inserted into the longitudinal incision, which passed across the stenosis into the non-coronary sinus in 14. A three-patch technique was used in 11 patients. Changes in aortic root following repair were documented in patients using both echocardiography and magnetic resonance imaging (MRI). RESULTS: There were no operative deaths. The mean preoperative gradient was 66+/-17 mmHg (range 50-100 mmHg), which decreased to 14+/-7 mmHg (range 4-18 mmHg) early postoperatively. The late mean gradient was 15+/-5 mmHg. There was no significant difference in the incidence of postoperative aortic regurgitation or gradient across the repair between two techniques according to the echocardiograms and MRI findings. CONCLUSION: According to our study, we cannot demonstrate any benefit in reconstructing the whole aortic root for discrete SAS. A single-patch technique is easy, safe and appears durable. [ABSTRACT FROM AUTHOR]

Neoplasms arising within urethral diverticula are rare. It is important to know if a diverticulum is filled by tumor, but traditional diagnostic methods, such as voiding cystourethrography, cannot detect a tumor or diverticulum, as in our case. We report an unusual case of leiomyoma developing in a female urethra diverticulum and review the literature. [ABSTRACT FROM AUTHOR]

Current COVID-19 mRNA vaccines delivered intramuscularly (IM) induce effective systemic immunity, but with suboptimal immunity at mucosal sites, limiting their ability to impart sterilizing immunity. There is strong interest in rerouting immune responses induced in the periphery by parenteral vaccination to the portal entry site of respiratory viruses, such as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), by mucosal vaccination. We previously demonstrated the combination adjuvant, NE/IVT, consisting of a nanoemulsion (NE) and an RNA-based RIG-I agonist (IVT) induces potent systemic and mucosal immune responses in protein-based SARS-CoV-2 vaccines administered intranasally (IN). Herein, we demonstrate priming IM with mRNA followed by heterologous IN boosting with NE/IVT adjuvanted recombinant antigen induces strong mucosal and systemic antibody responses and enhances antigen-specific T cell responses in mucosa-draining lymph nodes compared to IM/IM and IN/IN prime/boost regimens. While all regimens induced cross-neutralizing antibodies against divergent variants and sterilizing immunity in the lungs of challenged mice, mucosal vaccination, either as homologous prime/boost or heterologous IN boost after IM mRNA prime, was required to impart sterilizing immunity in the upper respiratory tract. Our data demonstrate the benefit of hybrid regimens whereby strong immune responses primed via IM vaccination are rerouted by IN vaccination to mucosal sites to provide optimal protection against SARS-CoV-2., Competing Interests: Declaration of interests P.T.W., M.S., J.R.B., and A.G.-S. are co-inventors on a patent for NE/IVT. The A.G.-S laboratory has received research support from GSK, Pfizer, Senhwa Biosciences, Kenall Manufacturing, Blade Therapeutics, Avimex, Johnson & Johnson, Dynavax, 7Hills Pharma, Pharmamar, ImmunityBio, Accurius, Nanocomposix, Hexamer, N-fold LLC, Model Medicines, Atea Pharma, Applied Biological Laboratories and Merck, outside of the reported work. A.G.-S. has consulting agreements with the following companies involving cash and/or stock: Castlevax, Amovir, Vivaldi Biosciences, Contrafect, 7Hills Pharma, Avimex, Pagoda, Accurius, Esperovax, Farmak, Applied Biological Laboratories, Pharmamar, CureLab Oncology, CureLab Veterinary, Synairgen, Paratus, Pfizer and Prosetta, outside of the reported work. A.G.-S. has been an invited speaker in meeting events organized by Seqirus, Janssen, Abbott, and Astrazeneca. A.G.-S. is inventor on patents and patent applications on the use of antivirals and vaccines for the treatment and prevention of virus infections and cancer, owned by the Icahn School of Medicine at Mount Sinai, New York. The M.S. laboratory received unrelated research support as sponsored research agreements from ArgenX BV, Phio Pharmaceuticals, 7Hills Pharma LLC and Moderna., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction/objectives: Controversy exists regarding the concordance of patient-reported outcome measures (PROMs) with other assessment parameters in systemic sclerosis-associated interstitial lung disease (SSc-ILD). This study aims to explore the association between the St. George's Respiratory Questionnaire (SGRQ) and various outcome measures in patients with SSc-ILD within a real-world cross-sectional setting., Method: Patients with SSc-ILD were consecutively recruited from our SSc cohort. Simultaneous administration of SGRQ, scleroderma Health Assessment Questionnaire (sHAQ), respiratory visual analog scale (R-VAS), pulmonary function tests (PFTs), and the 6-min walking test (6-MWT) was conducted. The total extent of lung fibrosis was quantified using high-resolution computed tomography (HRCT) images. Relationships between SGRQ and functional, radiographic, and other patient-reported outcome measures were analyzed., Results: The total SGRQ score demonstrated correlations with forced vital capacity (FVC) and R-VAS (r =  - 0.397, p = 0.016 and r = 0.418, p = 0.027, respectively). Symptom score correlated with ILD-extension (r = 0.430, p = 0.005); activity score correlated with FVC and R-VAS (r =  - 0.502, p = 0.002 and r = 0.395, p = 0.038, respectively); impact score correlated with R-VAS (r = 0.386, p = 0.043). In patients with fibrosis extent exceeding 20%, total SGRQ score was associated with sHAQ and R-VAS (r = 0.398, p = 0.049; r = 0.524, p = 0.021, respectively), activity score with R-VAS (r = 0.478, p = 0.038), and impact score with 6-MWT-D and R-VAS (r =  - 0.489, p = 0.034; r = 0.545, p = 0.016, respectively). The symptom score and activity score demonstrated optimal performance in identifying patients with interstitial lung disease (ILD) extent exceeding 20% and forced vital capacity (FVC) less than 70% (area under the curve [AUC] 0.799, p = 0.002, and AUC 0.792, p = 0.03, respectively)., Conclusions: Our study reveals varying degrees of correlation between SGRQ and distinct outcome measures. Given the incomplete alignment of SGRQ with other outcome measures, an integrative approach utilizing existing criteria as complementary tools is recommended. Key Points • Patient-reported outcome measures (PROMs) derive from patients' subjective evaluations of the impact of the disease on their daily activities, social interactions, and psychological well-being. • PROMs frequently serve as outcome measures in randomized controlled trials, yet conflicting findings have emerged in relation to primary outcomes. • This study aims to assess the appropriateness and interrelation of PROMs with both radiological and functional outcome measures, providing insight into the current state of our patients in a real-life context. The investigation delves into the compatibility of these measures with each other., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objective: This retrospective study aimed to conduct a comprehensive analysis of Turkish Systemic Lupus Erythematosus (SLE) patients of Caucasian ethnicity, focusing on their clinical, immunological, and therapeutic characteristics, damage accural and mortality., Patients and Methods: We carried out a retrospective assessment of 300 SLE patients diagnosed between 2001 and 2017 at Kocaeli University Rheumatology Clinic. Demographic data, clinical manifestations, immunological profiles, treatment approaches, disease-related damage, and survival information were collected., Results: The study population had a significant female predominance (89%) with a mean age of disease onset of 35.4 (SD:13.3) years. Hematological (72.6%) and mucocutaneous (72%) manifestations were the most common clinical findings, followed by arthritis (66.3%). Females had higher frequency of photosensitivity (p = 0.019), malar rash (p < 0.001), and alopecia (p = 0.014). Anti-dsDNA antibodies were detected in 61.6% of patients, while 57% of patients had hypocomplementemia. Secondary antiphospholipid syndrome was observed in 15.3% of patients; the most common manifestations included deep venous thrombosis (32.6%) and cerebrovascular accidents (30.4%). Lupus Nephritis (LN) affected 40.3% of the cohort. The most common pathologic finding was Class IV LN (30.5%). Eventually, 13 (4.3%) patients developed chronic kidney disease (CKD) and 4 had renal replacement therapies. Patients with LN had higher usage of pulse steroids, azathioprin, mycophenolate mofetil, cyclophosphamide, and rituximab (p < 0.001 for each).  In the juvenile-onset group (n = 31, 10.3%), an increased occurrence of malar rash (p = 0.009), nephritis (p = 0.034), hypocomplementemia (p = 0.001), positive anti-dsDNA (p = 0.007), anti-Sm (p = 0.046), anti-rib-P (p = 0.014) antibodies were observed. At least one damage parameter was observed in 32.6% patients with musculoskeletal manifestations being the most common. Thirteen patients were diagnosed with various malignancies, with cervical cancer being the most common (4 cases). The total 5 and 10-year survival rates were 92.5% and 86.7%, respectively. However, patients with CKD, had lower survival rates; 75% at 3 years and 60% at 15 years. Regression analysis demonstrated an association of CKD and  history of infections with decreased survival (p = 0.02, each)., Conclusion: Ethnicity and geography influence the clinical diversity of SLE. Recognizing these disparities is crucial for tailoring patient care. Future inception cohort studies in Turkish SLE patients are necessary to address the limitations of retrospective research., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Sjögren's syndrome (SS) is an autoimmune systemic disease affecting many organs and systems, such as genital system., Aim: This study aimed to present the gynecological symptoms of patients who were followed up in an outpatient clinic because of primary Sjögren's syndrome (pSS) and secondary Sjögren's syndrome (sSS) and to show how the disease affected sexuality., Methods: This study is a cross-sectional study conducted between 2019 and 2020. The study sample consisted of 60 pSS patients, 42 sSS patients, and 52 healthy control subjects., Outcomes: All the participants were questioned about sexuality, and completed the 36-item Short Form Survey, Hospital Anxiety and Depression Scale, Health Assessment Questionnaire, and Modified Hill questionnaire., Results: The patients had a mean age of 55.6 ± 11.85 years in pSS, 59.39 ± 11.18 years in sSS, and 56.1 ± 10.46 years in healthy control subjects. Vaginal and vulvar dryness and dyspareunia were present at a significantly higher rate in SS, especially in pSS, compared with the control subjects. The Health Assessment Questionnaire score was significantly lower in the pSS group than in the sSS group. Arthralgia, myalgia, and fatigue were prominent in all SS patients., Clinical Implications: Gynecological symptoms, sexual ability, and the effects of the disease on sexuality should be questioned in all SS patients., Strengths and Limitations: It is very important that we evaluate the gynecological symptoms of both pSS and sSS patients and the effect of the disease on these symptoms. The small number of patients and healthy control subjects is a limitation., Conclusion: The gynecological and musculoskeletal symptoms negatively affected sexuality in patients with pSS and sSS, and the negative effect of the disease on sexuality was more pronounced in the pSS group., (© The Author(s) 2024. Published by Oxford University Press on behalf of The International Society of Sexual Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: Radiological alterations in psoriatic arthritis (PsA) are an established phenomenon frequently observed throughout the disease course. Our goal was to investigate the changes in the bone structure of PsA patients by conventional radiography., Methods: This study designed as a retrospective cohort study and cross-sectional evaluation for disease activity. The disease activity and the severity of skin and nail involvement were assessed. The Simplified Psoriatic Arthritis Radiographic Score (SPARS) was used to investigate the radiological progression. Logistic regression analysis was used to determine the predictors of radiological changes., Results: Joint space narrowing and bone proliferation in hands (p = 0.001 and p = 0.001, respectively) and joint space narrowing in feet (p = 0.047) were more common at the final evaluation than at the baseline assessment. Total scores of joint space narrowing and bone proliferation in hands and feet were higher at the last visit than at the initial assessment (p < 0.001). Male gender (p = 0.030, OR 4.32 (95%CI 1.15-16.15)], older age (for joint space narrowing [p = 0.026 OR 1.08 (95%CI 1.01-1.56)] and for proliferation [p = 0.025 OR 1.08 (95%CI 1.01-1.44)]), high Disease Activity index for Psoriatic Arthritis (DAPSA) scores at baseline [p = 0.032 OR 6.21 (95%CI 1.17-32.92)], and symmetrical polyarticular involvement at baseline [p = 0.025 OR 5.3 (95% CI 1.23-22.4)] were found as predictors of structural changes., Conclusion: By the end of the decade, joint space narrowing and proliferation were observed to be more common than erosion. Male gender, older age, higher initial DAPSA scores, and initial polyarticular involvement were identified as predictors of radiological damage. Key Points • The radiological changes of Psoriatic arthritis are a well-known entity. However, studies investigating the progression of joint involvement over time are scarce. • This study reveals that joint space narrowing and proliferation are the most prominent radiological alterations in Psoriatic Arthritis patients at the end of the decade. • Male gender, older age, higher baseline DAPSA scores, and initial polyarticular involvement are predictive factors influencing the progression of bone destruction  in Psoriatic Arthritis patients., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objectives: Digital ulcers (DUs) are associated with a significant burden in systemic sclerosis (SSc) by leading to severe pain, physical disability, and reduced quality of life. This effort aimed to develop recommendations of the Turkish Society for Rheumatology (TRD) on the management of DUs associated with SSc., Methods: In the first meeting held in December 2020 with the participation of a task force consisting of 23 rheumatologists the scope of the recommendations and research questions were determined. A systematic literature review was conducted by 5 fellows and results were presented to the task force during the second meeting. The Oxford system was used to determine the level of evidence. The preliminary recommendations were discussed, modified, and voted by the task force and then by members of TRD via e-mail invitation allowing personalised access to a web-based questionnaire [SurveyMonkey®]., Results: A total of 23 recommendations under 7 main headings were formulated covering non-pharmacological measures for the prevention of DUs and pharmacological treatments including vasodilators, anti-aggregants, antibiotics, wound care, pain control, and interventions including sympathectomy, botulinum toxin, and surgery. Risk factors, poor prognostic factors, prevention of DU and adverse effects of medical treatments were reported as 4 overarching principles., Conclusions: These evidence-based recommendations for the management of SSc-associated DUs were developed to provide a useful guide to all physicians who are involved in the care of patients with SSc, as well as to point out unmet needs in this field.

Multiple FDA-approved SARS-CoV-2 vaccines currently provide excellent protection against severe disease. Despite this, immunity can wane relatively fast, particularly in the elderly and novel viral variants capable of evading infection- and vaccination-induced immunity continue to emerge. Intranasal (IN) vaccination more effectively induces mucosal immune responses than parenteral vaccines, which would improve protection and reduce viral transmission. Here, we developed a rationally designed IN adjuvant consisting of a combined nanoemulsion (NE)-based adjuvant and an RNA-based RIG-I agonist (IVT DI) to drive more robust, broadly protective antibody and T cell responses. We previously demonstrated this combination adjuvant (NE/IVT) potently induces protective immunity through synergistic activation of an array of innate receptors. We now demonstrate that NE/IVT with the SARS-CoV-2 receptor binding domain (RBD), induces robust and durable humoral, mucosal, and cellular immune responses of equivalent magnitude and quality in young and aged mice. This contrasted with the MF59-like intramuscular adjuvant, Addavax, which showed a decrease in immunogenicity with age. Robust antigen-specific IFN-γ/IL-2/TNF-α was induced in both young and aged NE/IVT-immunized animals, which is significant as their reduced production is associated with suboptimal protective immunity in the elderly. These findings highlight the potential of adjuvanted mucosal vaccines for improving protection against COVID-19., (© 2023. The Author(s).)

Recombinant protein-based SARS-CoV-2 vaccines are needed to fill the vaccine equity gap. Because protein-subunit based vaccines are easier and cheaper to produce and do not require special storage/transportation conditions, they are suitable for low-/middle-income countries. Here, we report our vaccine development studies with the receptor binding domain of the SARS-CoV-2 Delta Plus strain (RBD-DP) which caused increased hospitalizations compared to other variants. First, we expressed RBD-DP in the Pichia pastoris yeast system and upscaled it to a 5-L fermenter for production. After three-step purification, we obtained RBD-DP with > 95% purity from a protein yield of > 1 g/L of supernatant. Several biophysical and biochemical characterizations were performed to confirm its identity, stability, and functionality. Then, it was formulated in different contents with Alum and CpG for mice immunization. After three doses of immunization, IgG titers from sera reached to > 10 6 and most importantly it showed high T-cell responses which are required for an effective vaccine to prevent severe COVID-19 disease. A live neutralization test was performed with both the Wuhan strain (B.1.1.7) and Delta strain (B.1.617.2) and it showed high neutralization antibody content for both strains. A challenge study with SARS-CoV-2 infected K18-hACE2 transgenic mice showed good immunoprotective activity with no viruses in the lungs and no lung inflammation for all immunized mice., (© 2023. The Author(s).)

Purpose: The purpose of this study is to examine the prevalence of bullying among high school students, its associated factors, and the effects of engaging in bullying behaviors on mental health., Materials and Methods: This cross-sectional study was conducted between 2021 and 2022 in the province with the highest population of children in the Southeast Anatolia region of Turkey. The data of the study were collected using the Sociodemographic Characteristics Questionnaire prepared by the researchers, Olweus Bully/Victim Questionnaire (OBVQ), and Depression Anxiety Stress Scale 42 (DASS-42)., Results: Of the 880 students who participated in the study, 34.2% reported being a victim of bullying and 13.9% reported bullied someone. Regression analysis revealed that overweight/obesity was a risk factor for both victims and bullies. On the other hand, male gender and alcohol consumption were significantly associated with bullying. Scores for depression, anxiety, and stress were significantly higher in both the victim and the bullying groups compared to students who did not fall into this cycle., Conclusions: The present findings suggest that bullying in school creates problems not only for the bullying victims but also for the bullies. Health professionals assessing youth should be aware of the characteristics of young people who may be involved in bullying.

Objectives: The aim was to investigate the discriminative value of a wide range of quantitative computed tomography (qCT) parameters in systemic sclerosis (SSc) patients with and without pulmonary fibrosis (PF) and their association with pulmonary function tests (PFTs) and visual fibrosis scores (VFS)., Method: Thoracic high-resolution computed tomography (HRCT) images of SSc patients with and without PF were analyzed with Vitrea® Advanced Visualization software. The mean lung attenuation (MLA), skewness, kurtosis, and threshold-based volumes [low-density volume (LDV), medium-density volume (MDV), and high-density volume (HDV)] derived from the attenuation histograms of the right and left lungs were evaluated separately. Visual scores were measured semi-quantitatively and the overall extent of pulmonary parenchymal abnormality was calculated., Results: Forty-one SSc patients with PF (85.4% female; mean age 50.4 ± 15.6 years) were compared with 94 without PF (88.3% female; mean age 50 ± 11.5 years). All qCT parameters were significantly different between those with and without PF (p < 0.05). Amongst the qCT measurements, R-MLA, L-MLA, R-MDV, L-MDV, and left total lung volume (L-TLV) correlated with all three of forced vital capacity, carbon monoxide diffusion capacity, and VFS, even after adjustment for sex and age (|r|> 0.300 and p < 0.05). R-MLA, L-MLA, R-HDV/TLV, and L-HDV/TLV exhibited diagnostic accuracy in discriminating patients with PF (AUC value > 0.7)., Conclusion: QCT parameters differentiated SSc patients with PF from the ones without and showed a good correlation with VFS. With the application of user-friendly and less operator-dependent software, qCT analysis may become an objective tool for analysis of PF in SSc, complementary to PFTs and VFS. Key Points • Quantitative computed tomography parameters can accurately and objectively differentiate between SSc patients with and without PF. • Furthermore, in SSc patients with fibrosis, a moderate to a high correlation was identified between many of the qCT parameters, PFT results, and VFS., (© 2021. International League of Associations for Rheumatology (ILAR).)

Graves' disease (GD) and toxic multinodular goitre (TMNG) are the most common thyroid diseases which mainly lead to thyrotoxicosis, however, the underlying mechanism of distinct clinical presentations remains unclear. Protein extracts from the thyroid tissue specimens of the patients with GD and TMNG were subjected to Difference Gel Electrophoresis (DIGE). Differentially regulated protein spots were determined by image analysis, and the spots displaying statistically significant differences were identified by Matrix-Assisted Laser Desorption Ionization Time of Flight Mass Spectrometer (MALDI-TOF) followed by MASCOT search. Western blot analysis was used to verify changes occurring at the protein levels. The identified proteins were classified based on their functions in metabolic pathways using bioinformatics algorithms. Fifteen proteins showed significant alterations in abundance between the two disease groups. Bioinformatic analysis revealed the differentially regulated proteins were particularly related to catabolism, oxidative stress and especially energy utilization pathways, including glycolysis, proteolysis, ketone body catabolism and other energy metabolism-related pathways. SIGNIFICANCE OF THE STUDY: Previously, GD has been the subject of many studies that performed the proteomics approaches in the orbital tissue samples or tear. This is one of the very few studies that investigate the changes in the proteome of thyroid tissue in GD. We demonstrated mainly the upregulation of catabolic activity-related proteins in patients with GD compared to TMNG. Although it remains to be elucidated, some of these proteins can be used as markers for GD or have a role in the pathogenesis of the disease. Our study contributes the increasing data over time by providing new biomarker candidates for GD., (© 2021 John Wiley & Sons Ltd.)

Objectives: To investigate lung volume and density in patients with SSc and changes in these parameters because of PF, using a software-aided image quantification method, and compare this with a matched healthy control group., Methods: Thoracic high-resolution computed tomography (HRCT) images of patients and controls were analysed using Myrian XP Lung 3D software. Right, and left lung densities and volumes were calculated separately by a blinded operator. Results were analysed between subgroups to investigate associations with the clinical features., Results: A total of 135 patients with SSc and 38 healthy controls (HC) were included. Characteristics of the SSc patients were 94 (69.6%) without PF, 85.4% female, mean age 49.8 (15.4) years; 41 (30.4%) with PF, 88.3% female, mean age 50.2 (11.5) years, and HC group were 89.5% Female, mean age 52.2 (5.8) years. The right and left lung densities were significantly higher, and right and left lung volumes were significantly lower in the SSc patients with signs of fibrosis than those without and HC (P < .001 and P < .001; P = .006 and P = .002, respectively). After excluding patients with PF, right and left lung densities and volumes differed significantly between diffuse cutaneous SSc, limited cutaneous SSc, and HC (P = .002 and P < .001; P = .045 and P = .044, respectively). Patients who developed PF during follow-up had significantly lower baseline right and left lung densities than those who did not (P = .018; P = .014, respectively). Forced vital capacity and carbon monoxide diffusing capacity showed a weak correlation with lung densities and volumes in patients without PF and moderate to high correlation in PF patients., Conclusion: Lung density and volume in SSc patients changed significantly in those with PF and those without. Quantitative information extracted by soft-ware aided methods may contribute more to the detection, screening, and risk prediction in SSc-related PF., (© 2020 John Wiley & Sons Ltd.)

Objective: Familial Mediterranean Fever (FMF) is an autoinflammatory disease that is commonly present with recurrent episodes of fever, peritonitis, pleuritis or arthritis. Enthesitis and sacroilitis can also be seen in FMF. Spondylitis is a less common manifestation of joint involvement in FMF and there are controversial publications about whether this involvement is FMF-related or coincidentally. The aim of this study was to provide a comparison between ankylosing spondylitis (AS) patients and FMF patients with AS., Methods: A total of 404 patients who 360 of them was AS and 44 was FMF patients with AS (in accordance with Tel Hashomer) patients with AS (in accordance with modified New York criteria) were included in this study. All cases were evaluated retrospectively and patient's demographic and clinical data were recorded., Results: The mean age was 34.5 ± 8.6 years and 61.4% of patients were female in FMF group. In AS group, the mean age was 41.2 ± 10.8 years and 67.8% of patients were male. In AS group, 92% of patients had inflammatory back pain, 51% had hip pain, 30% had heel pain, 14% had peripheral arthritis and 11% had uveitis. In FMF group, 98% of patients had inflammatory back pain, 59% had hip pain, 48% had heel pain, 43% had peripheral arthritis and 4.5% had uveitis. Syndesmophyte and enthesitis on X-ray were seen in 18% and 22% of AS patients, and 7% and 41% of FMF patients with AS, respectively. There were significant differences between AS patients and FMF patients with AS in terms of heel pain (P: .017), peripheral arthritis (P: .000) and enthesitis (P: .006)., Conclusion: Peripheral arthritis and enthesitis were more frequent, and uveitis and syndesmophyte were less frequent in FMF patients with spondylitis than AS patients. When we look at gender differences, clinical and genetic features, it seems to be different condition from AS., (© 2020 John Wiley & Sons Ltd.)

Understanding the determinants of antibody specificity is one of the challenging tasks in antibody development. Monospecific antibodies are still dominant in approved antibody therapeutics but there is a significant body of work to show that multispecific antibodies can increase the overall therapeutic effect. Dual-specific or "Two-in-One" antibodies can bind to two different antigens separately with the same antigen-binding site as opposed to bispecifics, which simultaneously bind to two different antigens through separate antigen-binding units. These nonstandard dual-specific antibodies were recently shown to be promising for new antibody-based therapeutics. Here, we physicochemically and structurally analyzed six different antibodies of which two are monospecific and four are dual-specific antibodies derived from monospecific templates to gain insight about dual-specificity determinants. These dual-specific antibodies can target both human epidermal growth factor receptor 2 and vascular endothelial growth factor at different binding affinities. We showed that a particular region of clustered Vernier zone residues might play key roles in gaining dual specificity. While there are minimal intramolecular interactions between a certain Vernier zone region, namely LV4 and LCDR1 of monospecific template, there is a significant structural change and consequently close contact formation between LV4-LCDR1 loops of derived dual-specific antibodies. Although Vernier zone residues were previously shown to be important for humanization applications, they are mostly underestimated in the literature. Here, we also aim to resurrect Vernier zone residues for antibody engineering efforts., (© 2020 Wiley Periodicals LLC.)

Objectives: The Scleroderma Health Assessment Questionnaire (SHAQ) is a functional scale which consists of five scleroderma-specific items (overall disease severity, Raynaud's phenomenon, digital ulcers, respiratory and intestinal involvement) in addition to Health Assessment Questionnaire Disability Index (HAQ-DI). The objective of this study was to perform an adaptation and validation of a Turkish version of the SHAQ., Method: We validated psychometric properties of the scale with 70 consecutive systemic sclerosis (SSc) patients, who fulfilled the 2013 ACR/EULAR classification criteria for SSc. We evaluated test-retest reliability with the intraclass correlation coefficient (ICC), discriminant validity by stratifying patients according to organ involvements and disease subtypes, and convergent validity by testing the correlation between SHAQ and related components of Short Form 36 version 2 (SF-36v2). Internal consistency of the questionnaire was evaluated by Cronbach's alpha coefficient., Results: The SHAQ-global, visual analogue scales (VAS) of pulmonary, digital ulcer, and Raynaud's phenomenon were significantly correlated with the physical component score of the SF-36v2 (r = - 0.274, r = - 0.295, r = - 0.326, r = - 0.308, p < 0.05, respectively) for the convergent validity. The instruments could not discriminate between disease subtypes, except the digital ulcer VAS which was significantly higher in patients with dcSSc (1.00 ± 0.93 vs 0.55 ± 0.88, p = 0.026) for the discriminant validity. The HAQ-DI, SHAQ-global, digital ulcer VAS, and pulmonary VAS showed moderate correlation with an increase in the number of the organs involved (r = 0.319, r = 0.329, r = 0.341, r = 0.278, p < 0.05, respectively). We demonstrated high reproducibility for HAQ-DI (ICC = 0.962, 95% confidence interval = 0.934-0.978) and the other items of SHAQ. The overall internal consistency of the SHAQ was satisfactory (Cronbach's alpha = 0.953)., Conclusions: The Turkish version of the SHAQ met the requirements of validity and reproducibility.

Systemic sclerosis is associated with an increased prevalence/incidence of coronary artery disease. The aim of this study was to investigate epicardial adipose tissue (EAT) thickness which may contribute to cardio-metabolic risk in systemic sclerosis (SSc) patients without overt cardiac disease. EAT thickness was measured by transthoracic conventional Doppler echocardiography and compared in SSc patients (n = 47) and age- and sex-matched healthy controls (n = 36). The relationships between EAT thickness and markers of cardio-metabolic risk in SSc were examined. EAT thickness was significantly greater in patients with SSc compared to healthy controls (6 [7-5] vs 5 [6.75-3.25], p = 0.041). Compared to controls, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, neutrophil, B-type natriuretic protein (BNP), fasting plasma insulin and HOMA-IR were elevated (18 [31-10] vs 8.5 [18-4], p < 0.001; 0.4 [0.67-0.18] vs 0.21 [0.48-0.09], p = 0.012; 7510 [8731-5990] vs 6435 [7360-5195], p = 0.002; 4350 [5440-3570] vs 3390 [4168-2903], p < 0.001; 111 [185-74] vs 70 [127-70], p = 0.010; 6.7 [10.5-4.7] vs 4.7 [6.8-4.1], p = 0.008; 1.7 [2.6-1] vs 1.1 [1.7-0.9], p = 0.015, respectively). The total and low-density lipoprotein (LDL)-cholesterol were decreased in SSc patients (197 ± 45 vs 284 ± 36, p = 0.005; 118 [148-84] vs 140 [180-115], p = 0.003, respectively). In patients with SSc, the EAT thickness correlated positively with age, ESR, CRP, insulin, hemoglobin A1c and total and LDL-cholesterol (r = 0.574, p < 0.001; r = 0.352, p = 0.015; r = 0.334, p = 0.022; r = 0.290, p = 0.048; r = 0.317, p = 0.030; r = 0.396, p = 0.006 and r = 0.349, p = 0.016, respectively). Our study confirms that EAT thickness is greater in SSc patients compared to healthy controls using echocardiographic measurements. The results of our study suggest that EAT thickness is a candidate for atherosclerotic risk assessment in SSc.

Objective: To study the association of anti-Mullerian hormone (AMH) and small-dense low-density lipoprotein cholesterol (sd-LDL) with hepatosteatosis among young, lean, polycystic ovary patients., Study Design: A prospective, case control study was carried out including 79 young lean women. Fifty-eight women with polycystic ovary syndrome (PCOS) and 21 age-and BMI-matched healthy controls were recruited. Anthropometric variables, biochemical and hormonal parameters, insulin-resistance indices, lipid profiles including sd-LDL levels and serum AMH levels were determined. Hepatic lipid content was evaluated by abdominal ultrasonography (USG). Determining the best predictor(s) which discriminate normal USG and hepatosteatosis was analyzed by multiple logistic regression analyses. Adjusted odds ratios and 95% confidence intervals were also calculated., Results: PCOS patients had an increased prevalence of hepatosteatosis by 41.4% (P = 0.006) and they had significantly higher levels of sd-LDL and AMH when compared with the control group (P < 0.001). AMH and sd-LDL levels were positively and significantly associated with hepatosteatosis in young lean women with and without PCOS (OR: 2.877, 95%CI: 1.453-5.699, P: 0.02 and OR: 1.336, 95%CI: 1.083-1.648, P: 0.007, respectively). AMH and sd-LDL levels were positively correlated in PCOS patients (r = 0.626, P < 0.001). Both sd-LDL and AMH levels were the most predictive parameters for the determination of hepatosteatosis within the PCOS group. (OR: 3.347, 95%CI: 1.348-8.313, P = 0.009 and OR: 1.375, 95%CI: 1.072-1.764, P = 0.012, respectively). Statistically significant higher levels of AMH were associated with hepatosteatosis both in insulin resistance (IR) positive and IR negative PCOS patients (P < 0.001)., Conclusion: Hepatosteatosis is common in young lean PCOS patients. Increased AMH and sd-LDL levels may independently predict hepatosteatosis in young lean women with and without PCOS.

Electrode materials play a key role in enhancing the electricity generation in the microbial fuel cell (MFC). In this study, a new material (Ti-TiO(2)) was used as an anode electrode and compared with a graphite electrode for electricity generation. Current densities were 476.6 and 31 mA/m(2) for Ti-TiO(2) and graphite electrodes, respectively. The PCR-DGGE analysis of enriched microbial communities from estuary revealed that MFC reactors were dominated by Shewanella haliotis, Enterococcus sp., and Enterobacter sp. Bioelectrochemical kinetic works in the MFC with Ti-TiO(2) electrode revealed that the parameters by non-linear curve fitting with the confidence bounds of 95% gave good fit with the kinetic constants of η (difference between the anode potential and anode potential giving one-half of the maximum current density) = 0.35 V, K (s) (Half-saturation constant) = 2.93 mM and J (max) = 0.39 A/m(2) for T = 298 K and F = 96.485 C/mol-e(-). From the results observed, it is clear that Ti-TiO(2) electrode is a promising candidate for electricity generation in MFC.

Introduction: Paraovarian or paratubal cysts (PTCs) constitute about 10 % of adnexial masses. Although they are not uncommon; they rarely cause symptoms and are usually incidentally found. Actual incidence is not known. The symptoms occur when they grow excessively, or in case of hemorrhage, rupture or torsion., Methods: Here, literature review reporting the incidence, presentation and complications of PTCs is performed. Uncommon presentations of PTCs in three different cases, a giant PTC, torsion of PTC and borderline paratubal tumor, are also reported and discussed., Results: Ultrasonography, CT or MRI may be performed in preoperative evaluation; but none of these imaging techniques have specific criteria for diagnosis. So, in most cases misdiagnosis as an ovarian mass remains to be a problem., Conclusion: Paratubal cysts can become extremely big before causing symptoms. Torsion is another urgent issue regarding PTCs, necessiating urgent surgery for preservation of the ovary and the tube. Although malignancy is rare, borderline paratubal tumors have been reported in the literature.

Objectives: The aim of this study was to evaluate the nasal bone and bony nasal pyramid in adult Anatolian people., Method: A total of 80 patients (48 males, 32 females, mean age of 40.03 years) were all evaluated using CT. Upper, intermediate and inferior thickness of the nasal bone on each side and on the lateral and medial osteotomy line were measured. In addition, nasal bone length and pyriform aperture width were determined., Results: The bone thickness was 2.23 mm ± 0.15 mm in males and 2.19 mm ± 0.14 mm in females at the level of upper border of the nasal bone; 1.82 mm ± 0.32 mm in males and 1.81 mm ± 0.25 mm in females at the intermediate level; and 1.73 mm ± 0.30 mm in males and 1.86 mm ± 0.69 mm in females at the lower border of the nasal bone. The mean thickness on the lateral osteotomy line was 1.85 mm ± 0.32 mm in males and 1.91 mm ± 0.46 mm in females. The mean thickness of the medial osteotomy line was 2.08 ± 0.17 mm in males and 2.04 mm ± 0.17 mm in females. The mean length of the nasal bone was 30.61 mm ± 1.26 mm in males and 29.01 mm ± 1.12 mm in females. The mean width of the pyriform aperture was 18.83 mm ± 2.17 mm in males and 18.19 mm ± 1.85 in females., Conclusion: The dimensions of the nasal pyramid are known to be important in the selection of appropriate osteotome. Our results can be used for pre-operative evaluation of Anatolian people undergoing nasal surgery.

Objective: This study was planned to screen polycystic ovary syndrome (PCOS) women for albuminuria and to evaluate the association between urinary albumin excretion (UAE) and metabolic disturbances of PCOS. In addition, this is the first study in the literature evaluating the association between UAE and carotid intima-media thickness (CIMT) in PCOS cases., Study Design: The study population consisted of 65 PCOS women. The study was prospectively designed and performed in a university hospital. The diagnosis of PCOS was made according to the Rotterdam criteria: exclusion criteria were hyperprolactinemia, thyroid dysfunction, adrenal dysfunction, diabetes mellitus, hypertension, and pregnancy. Blood samples were collected in the follicular phase of a menstrual cycle and serum samples were analyzed for fasting glucose, insulin, and hormone and lipid profiles. Twenty-four hour urine specimens were collected for the detection of UAE. CIMT was estimated by visual assessment of the distance between the lumen-intima and intima-adventitia interfaces., Results: The mean age and BMI were 23 years and 23 kg/m(2), respectively. The median UAE was 7 mg/day (range: 0.3-154 mg/day). The median UAE as micrograms of albumin per milligram of creatinine (uACR) was 5.6 (0.28-159). Regarding the uACR cutoff value (>6.93 μg/mg), significantly higher levels of triglycerides, 17 OH-progesterone, insulin resistance (HOMA index > 2.1) and increased CIMT were present in these cases. Microalbuminuria (uACR > 25 μg/mg) was present in 6.2%. In the regression analyses serum HDL-C levels were found to be independent predictor for uACR > 2 μg/mg (OR: 0.85) and estradiol levels were the independent predicting factor for uACR > 6.93 μg/mg even after adjustments for age and BMI were performed (OR:1.02)., Conclusions: UAE, expressed as uACR > 6.93 μg/mg, seems to be an associated sign of metabolic problems which might help in discriminating PCOS at risk of future CVD. Further studies are needed before routine use of albuminuria in PCOS cases for the detection of CVD risk., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Congenital hypoplasia of the posterior arch of the atlas (C1), a developmental failure of chondrogenesis, is a rare anomaly and may range from partial clefts to total agenesis of the posterior arch. Ossification of the posterior arch usually occurs between the 3rd and 5th years of life. The incidence of posterior arch anomalies of the atlas is between 0.69% and 2.95%. For the evaluation of the patient, cervical lateral plain radiography, 2D or 3D reconstructed CT and MRI are very useful and important tools in initial diagnosis. Surgery is the treatment of choice in symptomatic compression. Excision of the posterior arch is performed. during surgery. After the surgery, patients may be followed up for instability and treated as necessary. A patient, admitted to the emergency department with head and neck trauma after a traffic accident is presented in this article. C1 hypoplasia was determined after detailed imagining studies and the radiology department consulted. When upper cervical anomalies are found in a young patient, the patient should be evaluated in detail with advanced radiological studies to avoid misinterpretation as fractures, luxation, osteolysis or instability. Consulting a radiologist could help making an accurate diagnosis and deciding on current therapeutic interventions.

The aim of this study was to evaluate ischemia-modified albumin levels (IMA) in polycystic ovary syndrome (PCOS) cases with and without insulin resistance and the correlation of IMA with carotid intima media thickness, homocysteine, and high-sensitivity C-reactive protein levels. Significantly higher levels of IMA in young lean PCOS cases, more relevant in insulin resistant cases, indicates chronic hypoxia and oxidative stress which might play a role in the metabolic consequences in PCOS., (Copyright © 2011 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

Batch experiments were conducted to investigate the thermophilic biohydrogen production using an enrichment culture from a Turkish hot spring. Following the enrichment, the culture was heat treated at 100 degrees C for 10 min to select for spore-forming bacteria. H(2) production was accompanied by production of acetate, butyrate, lactate and ethanol. H(2) production was associated by acetate-butyrate type fermentation while accumulation of lactate and ethanol negatively affected the H(2) yield. H(2) production was highest in the temperature range from 49.6 to 54.8 degrees C and optimum values for initial pH and concentrations of iron, yeast extract and glucose were 6.5, 40 mg/l, 4-13.5 g/l, respectively. PCR-DGGE profiling showed that the heat treated culture consisted of species closely affiliated to genus Thermoanaerobacterium.

Using magnesit (MgCO(3)) as a low cost source of magnesium ions in the struvite precipitation for the removal of high ammonium content of leachate was evaluated. Optimum molar concentration and pH conditions were analyzed to minimize the struvite solubility. Since solubility of magnesit in water is low, HCl was used to obtain soluble Mg. Maximum soluble Mg was obtained for the addition of 2 M HCl to the 1 M MgCO(3). Struvite precipitation with magnesit was effective for the removal of ammonium, suspended solid, phosphate and turbidity. Economical evaluation was made comparing the costs of two magnesium sources, MgCl(2) and MgCO(3). The economical analysis has shown that operation cost of struvite precipitation can be reduced about 18% by using MgCO(3) instead of MgCl(2). High salt concentration after struvite precipitation has no inhibitory effect on the anaerobic reactor performance.

Internal auditory canal (IAC) anomalies are rare malformations that may include alterations of shape, size or orientation of the IAC. Bilateral duplication of IAC is a very rare malformation and only one case has been described in literature. We report a case of bilateral duplicated IAC together with other inner ear anomalies.

A pneumocele is an abnormal dilatation of a paranasal sinus, most commonly affecting the frontal sinus. Although the etiology of pneumocele is not entirely known, several causative factors have been suggested including trauma, surgery, tumor and infection. We report here a case of post-traumatic pneumocele of the frontal sinus following a head trauma.

Removal of ammonium ion (NH(4)(+)) from leachate from Odayeri sanitary landfill, located in the European part of Istanbul, was examined using Gördes (Turkish) clinoptilolite. The effects of pH, flow rate, ammonium concentration and competitive cations on the removal efficiency were investigated in both batch and column studies. Thomas model were used to analyze the breakthrough data. Nonlinear least square method was applied for fitting the column data. The competitive effect was observed more effective in lower ammonium concentration and higher cation concentrations. Conditioning and regeneration using NaCl improved removal efficiency of ammonium from leachate.

Ammonium ion exchange from aqueous solution using clinoptilolite zeolite was investigated at laboratory scale. Batch experimental studies were conducted to evaluate the effect of various parameters such as pH, zeolite dosage, contact time, initial ammonium concentration and temperature. Freundlich and Langmuir isotherm models and pseudo-second-order model were fitted to experimental data. Linear and non-linear regression methods were compared to determine the best fitting of isotherm and kinetic model to experimental data. The rate limiting mechanism of ammonium uptake by zeolite was determined as chemical exchange. Non-linear regression has better performance for analyzing experimental data and Freundlich model was better than Langmuir to represent equilibrium data.

Background: Tuberous sclerosis (TS) is characterised by benign hamartomatous lesions in many organs. Diffusion tensor imaging (DTI) can detect microstructural changes in pathological processes., Objective: To determine apparent diffusion coefficient (ADC) and fractional anisotropy (FA) maps in children with TS and to investigate the diffusion properties in cortical tubers, white-matter lesions, perilesional white matter, and contralateral normal-appearing white matter, and to compare the results with ADC and FA maps of normal age- and sex-matched volunteers., Materials and Methods: Seven children and adolescents (age range 2-20 years) suffering from TS were included. MRI was performed on a 1.5-T scanner using a transmit/receive coil with T1-W and T2-W spin-echo and FLAIR sequences. DT images were acquired by using a single-shot echo-planar pulse sequence. Diffusion gradients were applied in six different directions with a b value of 1,000 s/mm(2)., Results: ADC was higher in cortical tubers than in the corresponding cortical location of controls. ADC values were higher and FA values were lower in white-matter lesions and perilesional white matter than in both the contralateral normal-appearing white matter of patients and in controls. There were no significant differences for both ADC and FA values in the normal-appearing white matter of patients with TS compared to controls., Conclusions: DTI provides important information about cortical tubers, white-matter abnormalities, and perilesional white matter in patients with TS.

We report a patient with intramedullary ruptured spinal dermoid tumour. The MR imaging revealed an intra-medullary lumbar mass heterogenous in intensity in all sequences. Fat droplets were observed in the subarachnoid space as well as in the dilated central spinal canal. Fat droplets in the subarachnoid space are frequently seen in the rupture of intraspinal dermoid tumours; however, fat droplets within the central canal is quite rare and was unexpected. Magnetic resonance imaging is a useful tool in the determination of spinal pathologies before they become large enough to cause severe symptoms and/or morbidity.

A patient with an intradiploic epidermoid cyst of calvarium was presented. Head injury that may cause the inclusion of epidermal cells into the diploe of the bone was present in his medical history. Skull radiographs and cranial magnetic resonance imaging (MRI) demonstrated the tumor. The tumor was totally removed and the patient was discharged free of symptoms.