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The presence of pneumocephalus without a history of intracranial or intrathecal procedures is a significant radiographic finding. Although pneumocephalus means a violation of the dural barrier or the presence of infection, intravascular pneumocephalus is different from intraparenchymal pneumocephalus and its benign nature must be known in the presence of intravenous catheterization. Herein, we present a case of iatrogenic intravascular pneumocephalus with CT findings. To our knowledge, there are only a few reported cases of iatrogenic intravascular pneumocephalus in the literature. Careful intravenous catheterization and diagnosis of the condition on imaging helps to prevent unnecessary treatment procedures. [ABSTRACT FROM AUTHOR]

Abstract Background Fever is an extremely common sign in paediatric patients and the most common cause for a child to be taken to the doctor. The literature indicates that physicians and parents have too many misconceptions and conflicting results about fever management. In this study we aim to identify knowledge, attitudes and misconceptions of primary care physicians regarding fever in children. Methods This cross-sectional study was conducted in April-May 2010 involving primary care physicians (n=80). The physicians were surveyed using a self-administered questionnaire. Descriptive statistics were used. Results In our study only 10% of the physicians knew that a body temperature of above 37.2°C according to an auxiliary measurement is defined as fever. Only 26.2% of the physicians took into consideration signs and symptoms other than fever to prescribe antipyretics. 85% of the physicians prescribed antipyretics to control fever or prevent complications of fever especially febrile seizures. Most of the physicians (76.3%) in this study reported that the height of fever may be used as an indicator for severe bacterial infection. A great majority of physicians (91.3%) stated that they advised parents to alternate the use of ibuprofen and paracetamol. Conclusions There were misconceptions about the management and complications of fever. There is a perceived need to improve the recognition, assessment, and management of fever with regards to underlying illnesses in children.

Abstract Background Sterols and Sphingolipids form lipid clusters in the plasma membranes of cell types throughout the animal and plant kingdoms. These lipid domains provide a medium for protein signaling complexes at the plasma membrane and are also observed to be principal regions of membrane contact at the inception of infection. We visualized different specific fluorescent lipophilic stains of the both sphingolipid enriched and non-sphingolipid enriched regions in the plasma membranes of live protoplasts of Arabidopsis thaliana. Results Lipid staining protocols for several fluorescent lipid analogues in plants are presented. The most emphasis was placed on successful protocols for the single and dual staining of sphingolipid enriched regions and exclusion of sphingolipid enriched regions on the plasma membrane of Arabidopsis thaliana protoplasts. A secondary focus was placed to ensure that these staining protocols presented still maintain cell viability. Furthermore, the protocols were successfully tested with the spectrally sensitive dye Laurdan. Conclusion Almost all existing staining procedures of the plasma membrane with fluorescent lipid analogues are specified for animal cells and tissues. In order to develop lipid staining protocols for plants, procedures were established with critical steps for the plasma membrane staining of Arabidopsis leaf tissue and protoplasts. The success of the plasma membrane staining protocols was additionally verified by measurements of lipid dynamics by the fluorescence recovery after photobleaching technique and by the observation of new phenomena such as time dependent lipid polarization events in living protoplasts, for which a putative physiological relevance is suggested.

To investigate the rate of colchicine use in the longitudinal follow-up of familial Mediterranean fever (FMF) carriers and identify variables that could predict the necessity of colchicine treatment in this group. The study was conducted in 9 pediatric rheumatology centers. The files of children with MEFV gene carriers were retrospectively reviewed between February 2014 and May 2024. The study included 869 children with a median follow-up duration of 28 months (12-124). In most of the cases (n: 369; 43.5%), MEFV gene analysis was ordered by a pediatric rheumatologist, while in 228 children (26.2%), gene analysis was conducted at the request of a geneticist. The most common reason for ordering MEFV gene analysis was the presence of FMF-like symptoms (n: 349; 40.1%), followed by genetic screening due to a family history of FMF in relatives (n: 267; 30.7%). Colchicine therapy was initiated in 13.9% (n: 121) of the children. Variables that showed statistically significant differences in colchicine users included having a family history of amyloidosis, the MEFV gene ordered by a pediatric rheumatologist, and the presence of FMF-like symptoms. Conclusions: A small number of MEFV gene carriers develop FMF symptoms during the follow-up period, most commonly within 2-3 years. We do not recommend routine family screening for the MEFV gene after the diagnosis of an index patient unless there is a history of amyloidosis in the family or individuals having FMF-like symptoms., Competing Interests: Declarations. Ethics approval: The study was approved by the local ethics committee of Zeynep Kamil Women and Children’s Diseases Training and Research Hospital (date: 20 March 2024, number: 38) and was performed according to the tenets of the Declaration of Helsinki. Written informed consent was received from the legal guardians of the children. Consent to participate: Informed consent was obtained from the legal guardians of the children. Consent for publication: Informed consent for publication was obtained from the legal guardians of the children. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Biobanking of tissue from clinically obtained kidney biopsies for later analysis with multiomic approaches, such as single-cell technologies, proteomics, metabolomics, and the different types of imaging, is an inevitable step to overcome the need of disease model systems and toward translational medicine. Hence, collection protocols that ensure integration into daily clinical routines by the usage of preservation media that do not require liquid nitrogen but instantly preserve kidney tissue for both clinical and scientific analyses are necessary. Thus, we modified a robust single-nucleus dissociation protocol for kidney tissue stored snap-frozen or in the preservation media RNAlater and CellCover. Using at first porcine kidney tissue as a surrogate for human kidney tissue, we conducted single-nucleus RNA sequencing with the widely recognized Chromium 10X Genomics platform. The resulting datasets from each storage condition were analyzed to identify any potential variations in transcriptomic profiles. Furthermore, we assessed the suitability of the preservation media for additional analysis techniques such as proteomics, metabolomics, and the preservation of tissue architecture for histopathological examination including immunofluorescence staining. In this study, we show that in daily clinical routines, the preservation medium RNAlater facilitates the collection of highly preserved human kidney biopsies and enables further analysis with cutting-edge techniques like single-nucleus RNA sequencing, proteomics, and histopathological evaluation. Only metabolome analysis is currently restricted to snap-frozen tissue. This work will contribute to build tissue biobanks with well-defined cohorts of the respective kidney disease that can be deeply molecularly characterized, opening up new horizons for the identification of unique cells, pathways and biomarkers for the prevention, early identification, and targeted therapy of kidney diseases. NEW & NOTEWORTHY In this study, we addressed challenges in integrating clinically obtained kidney biopsies into everyday clinical routines. Using porcine kidneys, we evaluated preservation media (RNAlater and CellCover) versus snap freezing for multi-omics processing. Our analyses highlighted RNAlater's suitability for single-nucleus RNA sequencing, proteome analysis and histopathological evaluation. Only metabolomics are currently restricted to snap-frozen biopsies. Our research established a cryopreservation protocol that facilitates tissue biobanking for advancing precision medicine in nephrology.

The aim of this study was to investigate the genotoxic effect of Propineb fungicide at different concentrations (0.167, 0.335 and 0.670 mg L -1 ) and different treatment times (24, 48, 72 and 96 h) on Danio rerio . At the end of the treatment periods, blood was collected from the fish with a heparin injector; smear preparations were prepared, fixed and stained. In the prepared preparations, the numbers of cells with MN and erythrocyte nucleus abnormalities were examined. It was found that propineb increased micronucleus formation at all treatment times and concentrations and induced the formation of erythrocytes with morphological abnormal nuclei such as segmented, kidney-shaped, notched, vacuolated nuclei and binucleated. The increase in micronucleus formation and the number of erythrocytes with abnormal nuclei were found to be concentration and treatment time-dependent. In conclusion, in this study, Danio rerio erythrocytes were used to evaluate the genotoxic effects of propineb fungicide on aquatic organisms, which have an important place in environmental risk assessment criteria. Since fungicides used in agricultural control such as propineb may have the potential to be genotoxic to aquatic organisms, the results of toxicity tests should be taken into consideration in the selection and use of concentrations of these chemicals.

The epidemiology of respiratory infections may vary depending on factors such as climate changes, geographical features, and urbanization. Pandemics also change the epidemiological characteristics of not only the relevant infectious agent itself but also other infectious agents. This study aims to assess the impact of the COVID-19 pandemic on the epidemiology of viral respiratory infections in children. We retrospectively reviewed the medical records of children aged ≤18 years with laboratory-confirmed viral respiratory infections other than COVID-19 from January 2018 to March 2023. Data on demographic characteristics, month and year of admission, and microbiological results were collected. During the study period, 1,829 respiratory samples were sent for polymerase chain reaction testing. Rhinovirus was identified in 24% of the patients, mixed infections in 21%, influenza virus in 20%, and respiratory syncytial virus in 12.5%. A 38.6% decrease in viral respiratory infections was observed in 2020, followed by a 188% increase in 2021. The respiratory syncytial virus was significantly more common in the post-pandemic period (13.8%) compared to the pre-pandemic period (8.1%), but no seasonal shift in respiratory syncytial virus infection was observed. There was also a yearly increase in influenza infections in the post-pandemic period compared to the pre-pandemic period. After the COVID-19 pandemic, the frequency of parainfluenza virus infections increased during the summer months, and this finding provides a new contribution to the existing literature.

Background: Education in end-of-life care (EoLC) is essential for improving the knowledge, attitudes and behaviours of intensive care nurses., Aim: This study aimed to evaluate the effect of online awareness education, based on the End-of-Life Nursing Education Consortium, on the knowledge levels, attitudes and behaviours of intensive care nurses., Study Design: This single-centre randomized controlled study. This study was performed in a tertiary intensive care unit in a university-affiliated hospital. Seventy-six intensive care nurses were selected and equally randomized. In the education, the group was given an EoLC awareness education programme on an online platform as 40-60-min sessions once a week for 4 weeks. The descriptive Information Form, EoLC Knowledge Test and the Nurses' Attitudes and Behaviours towards the EoLC Scale were used. In the study, intensive care nurses' knowledge, attitude and behaviour levels towards EoLC were evaluated before and after 4 weeks (±3 days) following the education., Results: When the mean post-education scores of the nurses in the education group were compared with the control group, it was found that their mean scores on the EoLC Knowledge Test (p = .001, Cohen's d = 1.354, 95% CI = 3.240-6.549), the attitude sub-dimension (p = .001, Cohen's d = 1.520, 95% CI = 4.510-7.858), the behaviour sub-dimension (p = .001, Cohen's d = 1.425, 95% CI = 3.206-6.214) and the total attitude and behaviour (p = .001, Cohen's d = 1.927, 95% CI = 8.408-13.643) were statistically significantly higher than those of the control group., Conclusion: This study showed that EoLC awareness education positively affected nurses' knowledge, attitudes and behaviours. Accordingly, planning EoLC awareness education online at regular intervals may be recommended for intensive care nurses., Relevance to Clinical Practice: Intensive care nurses may be given online EoLC awareness education to positively affect their knowledge, attitudes and behaviours about EoLC. Online EoLC education can be effective in terms of reaching a large number of nurses, time and cost., (© 2024 The Author(s). Nursing in Critical Care published by John Wiley & Sons Ltd on behalf of British Association of Critical Care Nurses.)

Objectives: To identify an optimal pediatric musculoskeletal ultrasound (MSUS) protocol for the detection of knee arthritis in patients with juvenile idiopathic arthritis (JIA) including a comparison with existing protocols. Secondary aims were to correlate MSUS-identified B-Mode (BM) and Power Doppler-Mode (PD) synovitis with clinical findings., Methods: Consecutive JIA patients with confirmed knee arthritis after clinical examination underwent a thorough MSUS study protocol which included views identified and consented by the Pediatric Rheumatology european Society (PReS) Imaging Working Party for the detection of synovitis. In total eight views including measurement of the suprapatellar recess were included. Scoring of synovitis followed the pediatric OMERACT criteria (BM and PD severity grading 0 to 3). Interobserver reliability of BM and PD was tested before study begin. Previously published MSUS protocols for knee synovitis were also identified from the literature and their scan protocols compared to identify differences in sensitivity for synovitis according to the number and specific type of views included. Finally, a clinically applicable MSUS protocol for knee synovitis could be proposed., Results: In 114 patients with clinically active knee inflammation, BM positivity (grading ≥ 1) was most frequently detected in the suprapatellar longitudinal and transverse scans performed in any positioning (frequency 97-99% in suprapatellar longitudinal in 30° or neutral respectively). PD positivity was however higher in these views performed in 30° flexion compared to neutral. Intrasynovial PD positivity (grading ≥ 1) was most frequently detected in the lateral parapatellar (69%, sensitivity 0.68, specificity 0.98), medial parapatellar (frequency 67%, sensitivity 0.67, specificity 1.0), the longitudinal lateral (68%, sensitivity 0.67, specificity 0.98) and suprapatellar transverse in 30° (frequency 64%, sensitivity 0.64, specificity 1.0). A combination of five views was the most sensitive for BM and PD synovitis. The suprapatellar recess size was analyzed by age and gender. For each group, the recess was wider in knees with arthritis than without (p < 0.001). Interobserver reliability of BM and PD positivity showed 85% agreement, with kappa 0.74 (very good). Three published studies with knee synovitis MSUS protocols were identified, which included a range of 1-3 views. Evaluation of the sensitivity of positive PD findings of each of these protocols reached a range of 53-83%; the highest sensitivity (91%) was achieved with the 5 views as identified by this study. These five views were therefore combined to form the Pediatric Internationally agreed Ultrasound (PIUS) knee protocol., Conclusion: BM and PD positivity reliably correlated with the identification of pathological findings in knees of patients with JIA. From an internationally agreed protocol of eight images, a combination of five showed the greatest sensitivity for synovitis. This protocol, termed 'PIUS-Knee' performed well when compared to existing protocols., (© 2024. The Author(s).)

In recent years, there have been unprecedented developments in artificial intelligence. Object detection, voice recognition, face recognition etc. are some of the artificial intelligence applications. In this study, an auxiliary method for the automatic detection of cracks, one of the main deterioration problems on highways, is proposed. The crack formation of hot mix asphalts is investigated with an image processing method modeled with Attention SegNet architecture. Styrene-butadiene-styrene (SBS), the most widely used additive in bitumen modification, was used at 2 %, 3 %, and 4 % ratios to modify 50/70 bitumen. Semi-circular asphalt specimens obtained with SBS modified bitumen were subjected to a semicircular bending (SCB) test and fracture performance was investigated. The effects of different temperature, notch size and additive on crack detection performance are evaluated. In the experimental study, maximum load, fracture energy, fracture toughness (KIC) values were obtained at low temperature, and resistance values against crack propagation were obtained by applying the J-integral method at intermediate temperature. The results demonstrated that with the addition of SBS, the fracture strength and maximum load values increased at each temperature value, with the 4 % SBS mixture offering the highest performance. Moreover, the image segmentation performed with SegNet provided high accuracy and precision values for cracks. It was observed that the accuracy values of the image processing methods decreased at low temperature, while at high temperature, higher accuracy values were obtained as the cracking rate., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

The continuous release of glucosinolates into the soil by Brassicaceae root exudation is a prerequisite to maintaining toxic levels of breakdown products such as isothiocyanates (ITCs). ITCs influence plant and microbial diversity in ecosystems, while fungi and Rhizobiaceae are particularly injured. Studies explaining the molecular mechanisms of the negative effects are presently limited. Therefore, we investigated the early effects of cyclic ITC goitrin on proteomes of the host and symbiotic Mesorhizobium loti in the nodules of Lotus japonicus and of free-living bacteria. In the nodules, many host proteins had a higher abundance, among them, peroxidases and pathogenesis-related PR-10 proteins functioning in the abscisic-acid-activated signaling pathway. In the microsymbiont, transporter proteins as a prominent group are enhanced; some proteins involved in N-fixation decreased. The proteomes give a report about the loss of immunity suppression resulting in the termination of symbiosis, which initiates nodule senescence. Free-living M. loti are severely damaged, indicated, i.a., by a decrease in transporter proteins, the assumed candidates for goitrin protein complex formation, and high proteolysis. The production of chicoric acid by the accompanying bacteria is inhibitory for M. loti but connected to goitrin elimination, as confirmed by mass spectrometric (MS) analysis. In summary, the nodulation process is severely affected by goitrin, causing nodule dysfunction and failed nodule development. N deficiency conditions leads to yellowish leaves and leaf abscission.

Pontocerebellar hypoplasia (PCH) is a heterogeneous group of neurodegenerative disorders characterized by hypoplasia and degeneration of the cerebellum and pons. We aimed to identify the clinical, laboratory, and imaging findings of the patients with diagnosed PCH with confirmed genetic analysis. We collected available clinical data, laboratory, and imaging findings in our retrospective multicenter national study of 64 patients with PCH in Turkey. The genetic analysis included the whole-exome sequencing (WES), targeted next-generation sequencing (NGS), or single gene analysis. Sixty-four patients with PCH were 28 female (43.8%) and 36 (56.3%) male. The patients revealed homozygous mutation in 89.1%, consanguinity in 79.7%, pregnancy at term in 85.2%, microcephaly in 91.3%, psychomotor retardation in 98.4%, abnormal neurological findings in 100%, seizure in 63.8%, normal biochemistry and metabolic investigations in 92.2%, and dysmorphic findings in 51.2%. The missense mutation was found to be the most common variant type in all patients with PCH. It was detected as CLP1 (n = 17) was the most common PCH related gene. The homozygous missense variant c.419G > A (p.Arg140His) was identified in all patients with CLP1. Moreover, all patients showed the same homozygous missense variant c.919G > T (p.A307S) in TSEN54 group (n = 6). In Turkey, CLP1 was identified as the most common causative gene with the identical variant c.419G > A; p.Arg140His. The current study supports that genotype data on PCH leads to phenotypic variability over a wide phenotypic spectrum., (© 2024. The Author(s).)

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) are the most common autoinflammatory syndromes in children. This study aimed to evaluate the clinical and laboratory parameters that may predict colchicine responsiveness.This retrospective, multicenter, cross-sectional study involved nine pediatric rheumatology centers from our country., The patients diagnosed with PFAPA were compared on the basis of their responses to colchicine. In the 806 (42.3% female 57.7% male) patients, the most common clinical findings were fever (100%), exudative tonsillitis (86.5%), pharyngitis (80.9%), and aphthous stomatitis (50.5%). The mean attack frequency was 13.5 ± 6.8 attacks per year lasting for a mean of 3.9 ± 1.1 days. Colchicine treatment was attempted in 519 (64.4%) patients, with 419 (80.7%) showing a favorable response. In patients who underwent MEFV gene analysis (70.8%), the most common variant was M694V heterozygous (16.8%). The presence of pharyngitis (p = 0.03, 95% CI 0.885 to 0.994), the presence of arthralgia (p = 0.04, 95% CI 0.169 to 0.958), and having more frequent attacks (p = 0.001, 95% CI 0.028 to 0.748) were found to be associated with colchicine unresponsiveness, whereas the carriage of the M694V variant (p = 0.001, 95% CI 0.065 to 0.242) was associated with colchicine responsiveness., Conclusion: This study identified the presence of pharyngitis, arthralgia, and increased attack frequency in patients with PFAPA as factors predicting colchicine unresponsiveness, whereas the carriage of the M694V variant emerged as a predictor of colchicine responsiveness. Predicting colchicine response at disease onset may facilitate a more effective management of PFAPA., What Is Known: • Colchicine treatment can be used in the prophylaxis of PFAPA disease. • Having the MEFV variant is the most commonly known factor in predicting response to colchicine., What Is New: • The presence of pharyngitis or arthralgia, and more frequent attacks in PFAPA disease were found to be independently associated with colchicine unresponsiveness. • Carrying the M694V variant was identified as the sole factor predicting colchicine responsiveness., (© 2024. The Author(s).)

Phenomenon: Physician immigration from other countries is increasing as developed countries continue to be desirable destinations for physicians; however, the determinants of Turkish physicians' migration decisions are still unclear. Despite its wide coverage in the media and among physicians in Türkiye, and being the subject of much debate, there is insufficient data to justify this attention. With this study, we aimed to investigate the tendency of senior medical students in Türkiye to pursue their professional careers abroad and its related factors. Approach: This cross-sectional study involved 9881 senior medical students from 39 different medical schools in Türkiye in 2022. Besides participants' migration decision, we evaluated the push and pull factors related to working, social environment and lifestyle in Türkiye and abroad, medical school education inadequacy, and personal insufficiencies, as well as the socioeconomic variables that may affect the decision to migrate abroad. The analyses were carried out with a participation rate of at least 50%. Findings: Of the medical students, 70.7% had emigration intentions. Approximately 60% of those want to stay abroad permanently, and 61.5% of them took initiatives such as learning a foreign language abroad (54.5%) and taking relevant exams (18.9%). Those who wanted to work in the field of Research & Development were 1.37 (95% CI: 1.22-1.54) times more likely to emigrate. The push factor that was related to emigration intention was the "working conditions in the country" (OR: 1.89, 95% CI: 1.56-2.28) whereas the "social environment/lifestyle abroad" was the mere pull factor for the tendency of emigration (OR: 1.73, 95% CI: 1.45-2.06). In addition, the quality problem in medical schools also had a significant impact on students' decisions (OR: 2.20, 95% CI: 1.83-2.65). Insights: Although the percentage of those who want to emigrate "definitely" was at the same level as in the other developing countries, the tendency to migrate "permanently" was higher in Türkiye. Improving working conditions in the country and increasing the quality of medical faculties seem vital in preventing the migration of physicians.

Secreted immune proteases "Required for Cladosporium resistance-3" (Rcr3) and "Phytophthora-inhibited protease-1" (Pip1) of tomato (Solanum lycopersicum) are both inhibited by Avirulence-2 (Avr2) from the fungal plant pathogen Cladosporium fulvum. However, only Rcr3 acts as a decoy co-receptor that detects Avr2 in the presence of the Cf-2 immune receptor. Here, we identified crucial residues in tomato Rcr3 that are required for Cf-2-mediated signaling and bioengineered various proteases to trigger Avr2/Cf-2-dependent immunity. Despite substantial divergence in Rcr3 orthologs from eggplant (Solanum melongena) and tobacco (Nicotiana spp.), minimal alterations were sufficient to trigger Avr2/Cf-2-mediated immune signaling. By contrast, tomato Pip1 was bioengineered with 16 Rcr3-specific residues to initiate Avr2/Cf-2-triggered immune signaling. These residues cluster on one side of the protein next to the substrate-binding groove, indicating a potential Cf-2 interaction site. Our findings also revealed that Rcr3 and Pip1 have distinct substrate preferences determined by two variant residues and that both are suboptimal for binding Avr2. This study advances our understanding of Avr2 perception and opens avenues to bioengineer proteases to broaden pathogen recognition in other crops., Competing Interests: Conflict of interest statement: None declared, except for J.K., whom received funding from industry during part of this study., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Society of Plant Biologists.)

Background: Nailfold videocapillaroscopy (NVC) is the primary diagnostic tool for the assessment of microcirculation in the pediatric population., Objective: To define and standardize age-specific normal NVC patterns in healthy children and adolescents., Methods: A cross-sectional observational multicentric study was conducted in 564 participants aged 5-17 years. Dino-Lite CapillaryScope 200 Pro Model MEDL4N Pro was performed at 200× magnification. Quantitative and qualitative NVC parameters were analysed separately for each age group and divided into four groups based on age categories., Results: Of the 564 healthy participants, 54.9% were female. A total of 1184 images and 3384 capillaries were analysed. Positive correlations were observed between age and capillary density (P < 0.001, R = 0.450, CI95% 0.398-0.503). There was also a positive correlation between age and arterial/venous, loop diameter and capillary length, whereas there was a weak negative correlation between intercapillary distance. However, no correlation was found between age and capillary width. In addition, capillary density was significantly lower in the 5-7 age group compared with the other patient groups. Arterial limb diameter was lower in the 5-7 age group, while venous limb diameter was significantly wider in the 15-17 age group compared with the other patient groups. Dilated capillaries (8.7%), capillary tortuosity (14.4%), crossed capillaries (43.1%), micro-haemorrhages (2.7%) and avascular area (4.8%) were present in all age groups. Excellent intra- and interobserver ICC values were obtained for all parameters., Conclusion: These findings hold potential significance for future studies, aiding in the analysis and differentiation of children suspected of rheumatological diseases with potential microangiopathy., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: To describe the clinical features and treatment outcomes of children with juvenile psoriatic arthritis (JPsA) and compare the distinct patterns of the disease between early-onset and late-onset age groups., Methods: Patients with JPsA followed regularly for at least 6 months between 2010 and 2020 in seven paediatric rheumatology centres in Turkey were included in the study. The demographic features, clinical manifestations, treatment strategies and outcomes of the patients were evaluated retrospectively., Results: A total of 87 (46 male/41 female) patients were included in the study. The mean age at diagnosis was 11.9 years (s.d. 4.5). Fifty-seven (65.5%) patients had psoriasis at the time of diagnosis and arthritis preceded psoriasis in 10 (11.5%) patients. Thirty (34.5%) patients had dactylitis, 28 (32.2%) had nail pitting, 36 (41.4%) had involvement of the small joints and 20 (23%) had enthesitis. Sacroiliitis was detected in 11 (12.6%) patients by MRI. ANA was positive in 35 (40.2%) patients. Twelve children (13.8%) were in the early-onset (<5 years) group. Uveitis and ANA positivity were more common in the early-onset group. Active joint counts and activity scores of our patients showed significant improvement at month 6 and at the last control compared with baseline., Conclusion: About one-third of patients with JPsA do not have psoriasis at the time of diagnosis. In some patients, no skin lesion is seen during the course of the disease. Children with PsA seem to display two different phenotypes. Younger children have a female predominance, ANA positivity and uveitis, while older children have more axial involvement., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration., Conclusions: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests., What Is Known: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring., What Is New: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Importance: Treating patients with chronic urticaria using omalizumab has been shown to be safe and effective in randomized clinical trials. Multinational studies on long-term omalizumab performance in chronic urticaria in clinical practice settings are lacking, especially on drug survival. Drug survival, which refers to the length of time that patients are treated with a specific drug, is a comprehensive outcome covering effectiveness, safety, and patient and physician preferences. Furthermore, little is known about the reasons and potential predictors for omalizumab discontinuation., Objective: To investigate omalizumab drug survival as well as reasons and potential predictors for discontinuation in a large, diverse population., Design, Setting, and Participants: This international multicenter cohort study was conducted at 14 Urticaria Centers of Reference and Excellence in 10 countries, including all patients with chronic urticaria from these centers who were ever treated with omalizumab., Main Outcomes and Measures: Drug survival analysis was performed to assess time to discontinuation. Patient characteristics and treatment protocols were investigated by Cox regression analysis to identify potential predictors for omalizumab discontinuation., Results: In 2325 patients with chronic urticaria who started omalizumab between June 2009 and July 2022, the mean (SD) age of the cohort was 42 (6) years, and 1650 participants (71%) were female. Overall omalizumab survival rates decreased from 76% to 39% after 1 to 7 years, respectively (median survival time, 3.3 [95 % CI, 2.9-4.0] years), primarily due to discontinuation from well-controlled disease in 576 patients (65%). Ineffectiveness and adverse effects were reasons for discontinuation in a far smaller proportion of patients, totaling 164 patients (18%) and 31 patients (4%), respectively. Fast treatment response was associated with higher rates of omalizumab discontinuation due to well-controlled disease (hazard ratio, 1.45 [95% CI, 1.20-1.75]), and disease duration of more than 2 years was associated with lower rates of discontinuation due to well-controlled disease (HR, 0.81 [95% CI, 0.67-0.98]). Immunosuppressive cotreatment at the start of omalizumab and autoimmune disease was associated with a higher risk for discontinuation due to ineffectiveness (HR, 1.65 [95% CI, 1.12-2.42]). The presence of spontaneous wheals (HR, 0.62 [95% CI, 0.41-0.93]) and access to higher dosages (HR, 0.40 [95% CI, 0.27-0.58) were both associated with a lower risk for discontinuation of omalizumab due to ineffectiveness., Conclusion and Relevance: This multinational omalizumab drug survival cohort study demonstrated that treatment of chronic urticaria with omalizumab in a clinical setting is effective and safe, and well-controlled disease is the main reason for treatment discontinuation. These findings on omalizumab drug survival rates and reasons and potential predictors for discontinuation may guide patients and physicians in clinical decision-making and expectation management. These results may call for the identification of biomarkers for chronic urticaria remission in complete responders to omalizumab treatment.

Background and Objectives: We aimed to ascertain the predictive power of the left atrial coupling index (LACI) in patients with end stage renal disease (ESRD) for heart failure with preserved ejection fraction (HFpEF). Materials and Methods: This is a retrospective study including 100 subjects between 18 and 65 years of age with ESRD and not on dialysis treatment. Patients were divided into groups with and without HFpEF. The LACI was defined as the ratio of the left atrial volume index (LAVI) to the a' wave in tissue Doppler imaging (TDI). Statistical analyses were performed, including univariate and multivariate regression analyses. Results: The mean age of the participants was 47 ± 13.3 years. Individuals with HFpEF exhibited a higher LACI. Univariate and multivariate regression analyses demonstrated that the predictive capacity of the LACI for HFpEF was considerably higher than that of the LAVI and other echocardiographic parameters. Conclusions: Higher LACI levels were consistently related to the presence of HFpEF in ESRD patients. The LACI can be easily obtained in daily practice using conventional Doppler echocardiographic measurements during left atrial functional assessments.

Background: This study aimed to review our institutional experience with the Fontan operation, the adverse severe events we encountered during mid-term follow-up, and the associated risk factors., Methods: In the study, the medical records of 40 patients (22 males, 18 females) who underwent Fontan operation between August 1993 and August 2020 were retrospectively reviewed. The patients were followed up for at least six months., Results: The Fontan operation was performed at a median age of 6.5 years (range, 3 to 22 years), and the mean follow-up time was 4.1±3.8 years (range, 0.5 to 17 years). The most frequently occurring defect was tricuspid atresia (45.0%). Fifteen (37.5%) patients experienced 24 late adverse events. Late complications and severe side effects, in order of frequency, were arrhythmia in eight (53.3%) patients, hypoxia in five (33.3%) patients, and ventricular dysfunction in three (20%) patients. While protein-losing enteropathy and mortality were each observed in two (13.3%) patients, Fontan failure, thromboembolic event, pulmonary arteriovenous fistulae, and ascites were each observed in one (6.6%) patient. When possible risk factors for late complications were examined, a statistical significance was not found., Conclusion: Life expectancy and quality of life of patients with Fontan circulation have increased with advances in surgical technique and increased management success. However, complications are not uncommon after the Fontan operation, and late events remain a significant problem. The results of our study indicate that in mid-term follow-up of patients who underwent Fontan surgery at our institution, although not statistically significant, those who underwent fenestration and those operated at a later age tended to experience more severe events and late complications., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish Society of Cardiovascular Surgery.)

Background: Hyperphenylalaninemia (HPA) is defined as blood phenylalanine (Phe) levels exceeding the normal values (>120 μmol/L or >2 mg/dL) and is caused by a deficiency in the enzyme phenylalanine hydroxylase (PAH). The widespread screening of Phe levels in newborn screening programs has led to a very high number of patients with HPA., Methods: The samples were collected at various ages, not at the point of diagnosis. Nine pterin derivatives, including isoxanthopterin, sepiapterin, xanthopterin, primapterin, biopterin, neopterin, 7,8-dihydrobiopterin, 7,8-dihydroneopterin, and tetrahydrobiopterin (BH4), were analyzed in different HPA classes in serum, dried blood spots (DBS), and urine samples. A total of 18 patients, including six classical phenylketonuria (PKU), eight BH4-responsive PKU, and four mild HPA patients, were included in the study., Results: Among the nine pterin derivatives measured, a significant increase was observed in the levels of isoxanthopterin, biopterin, and 7,8-dihydrobiopterin in serum, dried blood spots (DBS), and urine samples of patients with HPA compared to the control group. However, elevations in isoxanthopterin, biopterin, and 7,8-dihydrobiopterin were observed in all HPA groups, although the extent of elevation varied among the different disease groups. There were also significant differences between HPA subgroups among these high values., Conclusion: In this study, it might be suggested that pterin profiling shows promising potential for its effective utilization in the differential diagnosis of HPA. Pterin profiling demonstrated its efficacy in accurately categorizing patients into distinct subtypes. This approach offers several notable advantages, including the ability to simultaneously screen multiple HPA subtypes through a single test, establish disease decision limits for pterins, shorten the time required for HPA differential diagnosis, reduce the risk of misdiagnosis, and increase overall diagnostic accuracy. This study is the most comprehensive study examining the association between HPA pterin in the literature. In our study, samples obtained from BH4-responsive PKU patients were on treatment. This may have affected the results. Preliminary findings on pterin profiles may need to be replicated in a prospective cohort of samples collected at the time of diagnosis to confirm the results., Competing Interests: The authors have no conflicts of interest to declare., (© 2024 S. Karger AG, Basel.)

Purpose: We aimed to investigate the role of lung ultrasound (LUS) score in the closure of hemodynamically insignificant patent ductus arteriosus (PDA) and the clinical findings of the patients before and after closure., Methods: The study groups (107 preterm neonates under 34 gestational weeks) were classified as hemodynamically significant PDA (group 1), hemodynamically insignificant PDA with closure therapy (group 2), hemodynamically insignificant PDA without closure therapy (group 3), and no PDA group (group 4) based on the echocardiography. 6- and 10-region LUS scores were compared for each group., Results: There was a significant difference between groups 1 and 3 on first, third, and seventh days. In contrast, groups 1 and 2 had similar LUS scores on the first, third, and seventh days. There was a negative correlation between LUS scores on the first and third days and gestational age, birth weight, the first- and fifth-minute APGAR scores, and there was a positive correlation between aortic root to left atrium ratio, and PDA diameter/weight ratio., Conclusion: We observed that LUS scores in patients with hemodynamically insignificant PDA treated with closure therapy were similar to in patients with hemodynamically significant PDA. Thus, LUS score can have role in PDA closure in preterm neonates. However, more comprehensive studies are needed., (© 2024 Wiley Periodicals LLC.)

Objectives: Ischemia-modified albumin (IMA) is a new biochemical marker of ischemia. We aimed to search blood IMA levels in neonates with congenital heart defects., Study Design: During the study period, patients diagnosed with congenital heart disease and newborns with a diagnosis of hyperbilirubinemia as a control group were included in the study. IMA level was analyzed using the IMA absorbance unit (ABSU) method., Results: In total, 57 newborns with congenital heart disease requiring cardiac operation for the study group and 38 newborns for the control group were included. There was no difference between the two groups in terms of gender, mode of delivery, and weeks of gestation. The average IMA values in the control group were 0.19 ± 0.09 ABSU. The prepostoperative mean IMA values of the patient group were 0.22 ± 0.07 and 0.23 ± 0.07 ABSU, respectively. Comparison of the postoperative IMA with the mean of the control group was statistically significant. Preoperative and postoperative IMA values of patients who have died due to primary heart disease and surgical complications were 0.21 ± 0.07 (0.08-0.32) ABSU and 0.25 ± 0.06 (0.12-0.36) ABSU, respectively. IMA levels were not statistically different between the two groups., Conclusion: Hypoxia and ischemia in congenital heart disease in the newborn period both preoperatively and postoperatively were important in prognosis. IMA was higher in the postoperative group. Many comprehensive studies are important in terms of preventing complications and decreasing mortality and morbidity by commenting on prognosis., Key Points: · IMA is a new biochemical marker of ischemia.. · In the literature, there are no reports about the relation between congenital heart defects and IM.. · The exposure of CHD patients to hypoxia/asphyxia in the preintra and postoperative periods cause neurologic deficits., Competing Interests: None declared., (Thieme. All rights reserved.)

Objectives: The mitochondrial elongation factor Tu (EF-Tu), encoded by the TUFM gene, is a GTPase, which is part of the mitochondrial protein translation mechanism. If it is activated, it delivers the aminoacyl-tRNAs to the mitochondrial ribosome. Here, a patient was described with a homozygous missense variant in the TUFM [c.1016G>A (p.Arg339Gln)] gene. To date, only six patients have been reported with bi-allelic pathogenic variants in TUFM, leading to combined oxidative phosphorylation deficiency 4 (COXPD4) characterized by severe early-onset lactic acidosis, encephalopathy, and cardiomyopathy., Case Presentation: The patient presented here had the phenotypic features of TUFM-related disease, lactic acidosis, hypotonia, liver dysfunction, optic atrophy, and mild encephalopathy., Conclusions: We aimed to expand the clinical spectrum of pathogenic variants of TUFM., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

Objectives: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS)., Methods: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis., Results: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7)., Conclusions: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: SGLT2 (sodium-glucose cotransporter 2) inhibitors (SGLT2i) can protect the kidneys and heart, but the underlying mechanism remains poorly understood., Methods: To gain insights on primary effects of SGLT2i that are not confounded by pathophysiologic processes or are secondary to improvement by SGLT2i, we performed an in-depth proteomics, phosphoproteomics, and metabolomics analysis by integrating signatures from multiple metabolic organs and body fluids after 1 week of SGLT2i treatment of nondiabetic as well as diabetic mice with early and uncomplicated hyperglycemia., Results: Kidneys of nondiabetic mice reacted most strongly to SGLT2i in terms of proteomic reconfiguration, including evidence for less early proximal tubule glucotoxicity and a broad downregulation of the apical uptake transport machinery (including sodium, glucose, urate, purine bases, and amino acids), supported by mouse and human SGLT2 interactome studies. SGLT2i affected heart and liver signaling, but more reactive organs included the white adipose tissue, showing more lipolysis, and, particularly, the gut microbiome, with a lower relative abundance of bacteria taxa capable of fermenting phenylalanine and tryptophan to cardiovascular uremic toxins, resulting in lower plasma levels of these compounds (including p-cresol sulfate). SGLT2i was detectable in murine stool samples and its addition to human stool microbiota fermentation recapitulated some murine microbiome findings, suggesting direct inhibition of fermentation of aromatic amino acids and tryptophan. In mice lacking SGLT2 and in patients with decompensated heart failure or diabetes, the SGLT2i likewise reduced circulating p-cresol sulfate, and p-cresol impaired contractility and rhythm in human induced pluripotent stem cell-derived engineered heart tissue., Conclusions: SGLT2i reduced microbiome formation of uremic toxins such as p-cresol sulfate and thereby their body exposure and need for renal detoxification, which, combined with direct kidney effects of SGLT2i, including less proximal tubule glucotoxicity and a broad downregulation of apical transporters (including sodium, amino acid, and urate uptake), provides a metabolic foundation for kidney and cardiovascular protection., Competing Interests: Disclosures Dr Rinschen declares pending research funding from Novo Nordisk unrelated to this work. Over the past 12 months, Dr Vallon has served as a consultant for Lexicon and received speaker honoraria from AstraZeneca and grant support for investigator-initiated research from AstraZeneca, Boehringer Ingelheim, Gilead, Lexicon, Maze, Merck, and Novo-Nordisk. Dr Magnussen receives study-specific funding from the German Center for Cardiovascular Research (DZHK; Promotion of Women Scientists Programme; FKZ 81X3710112), the Deutsche Stiftung für Herzforschung, the Dr Rolf M. Schwiete Stiftung, NDD, and Loewenstein Medical unrelated to the current work. Dr Magnussen has received speaker fees from AstraZeneca, Novartis, Boehringer Ingelheim/Lilly, Bayer, Pfizer, Sanofi, Aventis, Apontis, and Abbott outside this work. Dr Dugourd and R. Fallegger report funding from Pfizer. Dr Saez-Rodriguez reports funding from GSK, Pfizer, and Sanofi and fees from Travere Therapeutics, Stadapharm, and Astex. Dr Hoxha served on advisory boards for Novartis, Morphosys AG, Sotio, and Argenx. The other authors declare no conflict of interest.

Heavy metals are now persistently present in living things' environments, in addition to their potential toxicity. Therefore, the aim of this study was to utilize D. melanogaster to determine the biological effects induced by different heavy metals including cadmium chloride (CdCl 2 ), copper (II) sulfate pentahydrate (CuSO 4.5 H 2 O), and silver nitrate (AgNO 3 ). In vivo experiments were conducted utilizing three low and environmentally relevant concentrations from 0.01 to 0.5 mM under single and combined exposure scenarios on D. melanogaster larvae. The endpoints measured included viability, reactive oxygen species (ROS) generation and genotoxic effects using Comet assay and the wing-spot test. Results indicated that tested heavy metals were not toxic in the egg-to adult viability. However, combined exposure (CdCl 2 +AgNO 3 and CdCl 2 +AgNO 3 +CuSO 4.5 H 2 O) resulted in significant genotoxic and unfavorable consequences, as well as antagonistic and/or synergistic effects on oxidative damage and genetic damage., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Agriculture commonly utilizes crop protection products to tackle infestations from fungi, parasites, insects, and weeds. Validamycin A, an inhibitor of trehalase, possesses antibiotic and antifungal attributes. Epidemiological evidence has led to concerns regarding a potential link between pesticide usage and neurodegenerative diseases. The fruit fly, Drosophila melanogaster, has been recognized as a reliable model for genetic research due to its significant genetic similarities with mammals. Here, we propose to use D. melanogaster as an effective in vivo model system to investigate the genotoxic risks associated with exposure to validamycin A. In this study, we performed a neurotoxic evaluation of validamycin A in D. melanogaster larvae. Several endpoints were evaluated, including toxicity, intracellular oxidative stress (reactive oxygen species), intestinal damage, larval behavior (crawling behavior, light/dark sensitivity assay, and temperature sensitivity assay), locomotor (climbing) behavior, and neurogenotoxic effects (impaired DNA via Comet assay, enhanced by Endo III and formamidopyrimidine DNA glycosylase [FPG]). The results showed that exposure to validamycin A, especially at higher doses (1 and 2.5 mM), induced DNA impairment in neuroblasts as observed by Comet assay. Both larvae and adults exhibited behavioral changes and produced reactive oxygen species. Most importantly, this research represents a pioneering effort to report neurogenotoxicity data specifically in Drosophila larval neuroblasts, thus underscoring the importance of this species as a testing model in exploring the biological impacts of validamycin A. The in vivo findings from the experiments are a valuable and novel addition to the existing validamycin A neurogenotoxicity database., (© 2023 The Authors. Journal of Applied Toxicology published by John Wiley & Sons Ltd.)

Background: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients., Methods: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed., Results: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness., Conclusions: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood., (© 2023. The Author(s).)

Risky sexual behaviors of young women cause significant reproductive health problems. Therefore, it is vital to evaluate the sexual attitude and behavior characteristics of young women, their changes over the years, and the effects of information sources such as family, media, and friends, which play an effective role in bringing about changes in sexual behavior of young women. This study was planned to evaluate young women's premarital sexual behaviors and related factors in Turkiye between 01.11.2021 and 01.05.2022. It is designed as a descriptive correlation study conducted with 1.430 young women studying at universities in 30 metropolitan cities in Turkiye. The study data were collected using the "Participant Information Form" and the "Premarital Risky Sexual Behavior Assessment Scale for Young Women." This study showed that parents' educational status and geographical region was related to the young women's mean score and the subdimension score of the Premarital Sexual Behavior Assessment Scale for Young Women (PSAS-YW) ( p  < .001). Furthermore, young women's top source of information about sexuality was internet media (80 percent, n  = 1144), and the least source was their fathers (2.9 percent, n  = 41). There is a relationship between where young women spend most of their lives, the geographical region where their families live, and the risk of sexual behavior. The study observed that the geographical region where young women's parents live was effective in risky sexual behavior. While less risky sexual behavior was observed, primarily in the country's east, this rate increased in the western parts. Furthermore, while it was determined that young people mainly acquire sexual information through the media-internet or friends, it was observed that the participants in this group had the highest rate of risky sexual behavior. The age variable explained only 2 percent of the change in risky sexual behavior, and there was a linear increase between the mother's education level and the risky sexual behavior score of young women.

Objectives: Biological drugs are one of the most effective treatment methods for systemic juvenile idiopathic arthritis (SJIA) and can significantly prevent morbidity and mortality. This study aimed to evaluate the efficacy and safety of biologics in patients with SJIA and provide real-life data that might help improve the outcomes., Methods: TURSIS was a retrospective multicentre study carried out in patients with SJIA for whom a biological treatment had been initiated between 1st March 2013 and 30th December 2018. Data include patients' characteristics, laboratory-clinical results, outcomes, and safety-related variables. The 24-month follow-up data of the patients and the efficacy and safety of biological drugs were evaluated., Results: 147 patients were enrolled. The clinical course of the disease was as follows; it was monocyclic in 38.1%, polycyclic in 49%, and persistent in 12.9% of patients. First-choice biologics were interleukin (IL)-1 blockers in the majority of patients (56.5%), followed by the anti-IL-6 (25.2%) and anti-TNF-alpha drugs (18.4%). Anakinra was the most preferred biologic agent in patients with macrophage activation syndrome (MAS), and tocilizumab was used more frequently in patients with persistent type (p=0.000 and p=0.003). The most frequent switch rate was seen in patients receiving anakinra (n=40/68, 58.8%), and it was most frequently switched to canakinumab (n=32/40, 80%). Better physician's global assessment scores were achieved in patients treated with anakinra in Month 3, compared to other treatments (p=0.04)., Conclusions: The results of our study support the efficacy of biological drugs in particular anti-IL-1 and anti-IL-6 drugs, in the treatment of SJIA. These treatments resulted in improvement in activity of disease and provide a considerable decrease in the frequency of MAS.

Background: Mediastinal lymph node metastasis is an important prognostic factor in non-small cell lung cancer (NSCLC) patients without distant metastases. 18 F-2-fluoro-2-deoxy-Dglucose Positron Emission Tomography/Computed Tomography ( 18 F-FDG PET/CT) is recommended for detecting and staging lymph nodes and distant metastases in NSCLC patients., Objective: This study aims to investigate whether maximum standardized uptake (SUVmax), mean standardized uptake (SUVmean), metabolic tumor volume (MTV), and tumor lesion glycolysis (TLG) values of the primary tumor measured by 18 F-FDG PET/CT in resectable NSCLC can predict preoperative lymph node metastasis., Methods: This retrospective study included eighty NSCLC patients who underwent preoperative Positron Emission Tomography/Computed Tomography (PET/CT) for diagnosis and staging. The patients were stage I-III and had no distant metastases. Tumor metabolic parameters such as SUVmax, SUVmean, MTV, and TLG at PET/CT imaging were measured for preoperative diagnosis and staging, and the postoperative pathology results of the patients were examined. The pathology results divided patients with and without lymph node metastasis into two groups. The groups were compared with the student's t-test and chi-square test regarding 18 F-FDG PET/CT tumor metabolic parameters and other parameters., Results: Fifteen (18.8%) patients were female, and 65 (81.3%) were male. According to the postoperative pathology results, while 30 (37.5%) patients had lymph node metastasis, 50 (62.5%) did not. There was a significant difference between the groups regarding tumor SUVmax and SUVmean values (p = 0.036, p = 0.045). Overall survival in the N0 group was significantly higher than in the N1 + N2 group (p = 0.034); median survival was 30.2 months in N0 cases and 27.3 months in N1 and N2 groups., Conclusion: SUVmax and SUVmean values are significantly higher in patients with lymph node metastases than in patients without lymph node metastases, and this finding may provide useful information for predicting lymph node metastasis in patients with resectable NSCLC., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Aim: The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases., Materials and Methods: Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups., Results: The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis (n = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 (p < 0.05). Fever and abdominal pain were more frequently detected in Group 2 (p < 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups., Conclusion: To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)