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Objective: The Turkish Society of Pediatric Hematology set up a National Hemoglobinopathy Registry to demonstrate the demographic and disease characteristics of patients and assess the efficacy of a hemoglobinopathy control program (HCP) over 10 years in Turkey. Materials and Methods: A total of 2046 patients from 27 thalassemia centers were registered, of which 1988 were eligible for analysis. This cohort mainly comprised patients with β-thalassemia major (n=1658, 83.4%) and intermedia (n=215, 10.8%). Results: The majority of patients were from the coastal areas of Turkey. The high number of patients in Southeastern Anatolia was due to that area having the highest rates of consanguineous marriage and fertility. The most common 11 mutations represented 90% of all β-thalassemia alleles and 47% of those were IVS1-110(G->A) mutations. The probability of undergoing splenectomy within the first 10 years of life was 20%, a rate unchanged since the 1980s. Iron chelators were administered as monotherapy regimens in 95% of patients and deferasirox was prescribed in 81.3% of those cases. Deferasirox administration was the highest (93.6%) in patients aged

The purpose of this study was to research the problem of hemoglobin H (HbH) disease, to reveal the distribution patterns among different health centers, and to emphasize the importance of this disease for Turkey. A total of 273 patients were included from 8 hemoglobinopathy centers. The Antakya Hemoglobinopathy Center reported 232 patients and the remaining 7 centers reported 41 patients. PubMed was also searched for published articles related to Turkish patients with HbH disease, and we found 16 articles involving a total of 198 HbH patients. Most of the patients were reported from Antakya; thus, special attention should be paid to this region. This is a preliminary study to investigate the extent of the problem of HbH disease and it emphasizes the need for hematology associations or the Ministry of Health to record all cases of HbH disease in Turkey.

Objective: Cardiac failure due to iron overload remains the most common cause of death in patients with beta-thalassemia major. This study aimed to evaluate myocardial function in children with beta-thalassemia major using standard echocardiography technique and strain rate imaging. Materials and Methods: Conventional echocardiographic analysis, tissue velocity imaging, and strain/strain rate imaging of the left ventricle were evaluated in 48 children with beta thalassemia major (19 girls, 29 boys; 8.39+-4.05 years) and 22 healthy children (11 girls, 11 boys; 8+-3.72 years). Results: Conventional echocardiographic examinations revealed that beta-thalassemia patients had larger left ventricular end-systolic diameter, end-diastolic and end-systolic volume, left ventricular mass index, and mitral early/late diastolic flow velocity ratio (p

Objective: This study analyzes the clinical and laboratory findings of children with primary hemophagocytic lymphohistiocytosis (HLH) followed in various referral centers of Turkey.Materials and Methods: A simple three-page questionnaire prepared by the Turkish Histiocyte Study Group was used for documentation of patient data.Results: Age at diagnosis varied from 0.6 to 78 months (median±SD, 16.5±26.1). Sex distribution was almost equal (F/M=10/12). The frequencies of parental consanguinity and sibling death in the family history were 100% and 81.1%, respectively. The most common clinical findings were hepatomegaly (100%) and fever (95%). The most common laboratory findings were anemia (100%), hyperferritinemia (100%) and thrombocytopenia (90.9%). Triglyceride and total bilirubin levels in the deceased versus surviving group appear to be high (triglyceride: 394±183 mg/dl, 289±7 mg/dl; total bilirubin: 2.7±6.9 mg/dl, 0.5±1.2 mg/dl, respectively).Conclusion: We concluded that fever, hepatosplenomegaly, anemia, thrombocytopenia, and hyperferritinemia are the most common clinical and laboratory findings in primary HLH. Increased triglyceride and total bilirubin level at the time of diagnosis might be an indicator of poor prognosis in HLH.

Wiskott-Aldrich syndrome (WAS) is a clinical condition characterized by thrombocytopenia, eczema, and life-threatening infections. In some cases autoimmunity-related problems and even malignancy might be seen; however, some patients have milder clinical manifestations due to mutations in the same gene family, such as in X-linked thrombocytopenia (XLT), which is generally not associated with serious symptoms of disease, except for thrombocytopenia. Herein we report 2 siblings with chronic thrombocytopenia that were diagnosed with XLT based on a missense mutation in the WASP gene (223G>A, Val75Met). To the best of our knowledge this mutation has not been previously reported in a Turkish patient with XLT.

Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. It is difficult to differentiate between primary and secondary HLH based on clinical findings at the onset of disease. We aimed to find parameters that can help to differentiate primary and secondary HLH at initial diagnosis especially for physicians working in developing countries. Patient and Method: We retrospectively analyzed data of 38 HLH patients who were admitted to the Pediatric Hematology Department of Gaziantep University between January 2009 and December 2013. Results: Of 38 patients, 20 were defined as primary and 18 were secondary HLH. The average age of primary and secondary HLH patients was 31±9 and 81±14 months, respectively (p=0.03). We found consanguinity rates significantly higher in primary HLH patients compared to secondary HLH patients (p=0.03). We found that total and direct bilirubin levels significantly increased in primary HLH patients compared to secondary HLH patients (p=0.006, p=0.044). Also, CRP levels were found markedly increased in secondary HLH patients compared to primary ones (p=0.017). Conclusion: We showed that cholestasis and hyperbilurubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH and increased level of CRP should be considered in favor of secondary HLH.

Objective: Circumcision in patients with hemophilia is a controversial issue in medical professionals and it is affected from place of residence with different social, cultural and religious beliefs. Materials (Subjects) and Methods: In this study circumcision of 20 hemophiliac children were described with the familial approach to circumcision. All of the patients were circumcised by surgical technique after factor replacement and parents were evaluated with a questionnaire. Most of them thought that circumcision is an essential procedure in socio-cultural, psychological and religious aspects in Gaziantep, a city in southeast region of Turkey. Results: All of the patients were circumcised succesfully and most of the parents (between % 85-100) worry in religious and social aspects like exclusion, prevention and disabilities for their children as being non-circumcised. Conclusion: Circumcision is an essential procedure even in hemophilia in sociocultural, psychological and religious aspects in Gaziantep, a city in southeast region of Turkey for the patients and families. As being specialists for these patients we should perform the procedure with a team including surgeon, laboratory and hematologist to overheld these issues.

We present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS). The diagnosis of DDMS was based on findings including left facial asymmetry, left hemiparesis, mental retardation, right cerebral hemiatrophy, dilatation of the ipsilateral lateral ventricle and calvarial thickening. The diagnosis of MS was based on clinical findings including tall stature, myopia, retinitis pigmentosa, blue scleras, scoliosis, pectus excavatum, arachnodactyly and low ratio of upper/lower body segment. The patient developed hepatosplenomegaly, gingival hypertrophy and pancytopenia. Peripheral blood film and bone marrow examination showed that most of nucleated cells were blasts; immunophenotype of those cells showed CD11+, CD13+, CD14+, CD33+ and HLA-DR+. These findings confirmed the diagnosis of AML (FAB-M5). After induction chemotherapy, remission was obtained. To the best of our knowledge, our case is the third report of AML in MS syndrome, while AML associated with DDMS and MS has not been previously reported in the literature.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. Micro RNAs (miRNA) are about 22 nucleotide-long, small RNAs encoded with genes, and they have regulatory functions in immune response. Objective: To determine the miRNA expression levels of 11 secondary HLH patients, we evaluated the associations of miRNA levels with pathogenesis, clinical presentation, and prognosis of the disease. Patients and Methods: Patients who were diagnosed with secondary HLH from January 2011 to December 2012 were included in this study. We profiled the expressions of 379 miRNAs in plasma of both HLH patients and healthy controls. Patients were evaluated regarding with age, clinical findings, miRNA expresions, laboratory data, treatment, and prognosis, by using descriptive statistics. Results: A total of 11 secondary HLH patients and 11 healthy children were included in this study. miR-205-5p was expressed in all case and controls and expression level of miR-205-5p was found 6.21 fold higher than control group (p=0.01). We detected the second highest expression percent in miR-194-5p with 81% of cases and controls. Expression level of miR-194-5p was found to have 163 fold higher than controls (p= 0.009). miR-30c-5p showed 77% expression percent in cases and controls together. The expression level of this miRNA was detected 9 fold decreased in HLH patients compared to healthy children (p= 0.031). Conclusion: We showed that miR-205-5p, miR-194-5p and miR-30c-5p could be useful plasma biomarkers for HLH. Further research is needed in larger and homogenous study groups, especially for these miRNAs as biomarkers for HLH.

This paper proposes a streamlined approach to addressing the problem of allocating finite crew resources to concurrent jobs in the context of municipal drainage infrastructure maintenance. The problem was defined from the perspective of a project manager involved in planning such operations on a day-by-day basis. The problem statement was then transformed into a simplified Integer Linear Programming optimization model. Performance metrics were devised to evaluate the optimization model’s effectiveness. A heuristic algorithm representing the decision-making process by a seasoned planner in the partner company was also developed. Both methods were applied to a case study and contrasted based on the same performance metrics. The findings underscored substantial optimization benefits in rendering decision support in resource-constrained drainage construction operations planning. In conclusion, this research presents an alternative strategy for navigating the complexities inherent in finite crew resource allocation on multiple concurrent drainage projects; lends a cost-effective optimization solution to improving the utilization of finite available crews while satisfying service demands from multiple clients to the largest extent possible.

Abstract The electrocardiogram (ECG) is a fundamental and widely used tool for diagnosing cardiovascular diseases. It involves recording cardiac electrical signals using electrodes, which illustrate the functioning of cardiac muscles during contraction and relaxation phases. ECG is instrumental in identifying abnormal cardiac activity, heart attacks, and various cardiac conditions. Arrhythmia detection, a critical aspect of ECG analysis, entails accurately classifying heartbeats. However, ECG signal analysis demands a high level of expertise, introducing the possibility of human errors in interpretation. Hence, there is a clear need for robust automated detection techniques. Recently, numerous methods have emerged for arrhythmia detection from ECG signals. In our research, we developed a novel one‐dimensional deep neural network technique called linear deep convolutional neural network (LDCNN) to identify arrhythmias from ECG signals. We compare our suggested method with several state‐of‐the‐art algorithms for arrhythmia detection. We evaluate our methodology using benchmark datasets, including the PTB Diagnostic ECG and MIT‐BIH Arrhythmia databases. Our proposed method achieves high accuracy rates of 99.24% on the PTB Diagnostic ECG dataset and 99.38% on the MIT‐BIH Arrhythmia dataset.

The increasing severity of cyber-attacks against organizations emphasizes the necessity for efficient threat intelligence. This article presents a novel multi-layered architecture for threat intelligence that integrates diverse data streams, including corporate network logs, open-source intelligence, and dark web monitoring, to offer a comprehensive overview of the cybersecurity threat landscape. Our approach, distinct from previous studies, uniquely integrates these varied features into the machine-learning algorithms (XGBoost, Gradient Boosting, LightGBM, Extra Trees, Random Forest, Decision Tree, K-Nearest Neighbor, Gaussian Naive Bayes, Support Vector Machine, Linear Discriminant Analysis, Logistic Regression, ridge Classifier, AdaBoost and Quadratic Discriminant Analysis) using various feature selection algorithms (information gain, correlation coefficient, chi-square, fisher score, forward wrapper, backward wrapper, Ridge classifier) to enhance real-time threat detection and mitigation. The practical LITNET-2020 dataset was utilized to evaluate the proposed architecture. Extensive testing against real-world cyber-attacks, including malware and phishing, demonstrated the robustness of the architecture, achieving exceptional results. Specifically, XGBoost demonstrated the highest performance with a detection accuracy of 99.98%, precision of 99.97%, and recall of 99.96%, Significantly surpassing traditional methods. Gradient Boosting and LightGBM also exhibited excellent performance, with accuracy, precision, and recall values of 99.97%. Our findings underscore the effectiveness of our architecture in significantly improving an organization’s capability to identify and counteract online threats in real-time. By developing a comprehensive threat intelligence framework, this study advances the field of cybersecurity, providing a robust tool for enhancing organizational resilience against cyber-attacks.

The purpose of this research was to design a wisdom process model with an emphasis on narrative and Qur'anic teachings from Islamic sources. The method of this research was a qualitative type of Grounded Theory, and in order to check content validity, the descriptive contextualization method was used. With the formation of the semantic domain, the concepts corresponding to wisdom were identified in Islamic sources and analyzed in four stages of open, focused, central and selective coding. The result of this stage was the achievement of 1276 open codes, 344 focused codes, 84 central codes, 19 categories and 10 main micro components. These components are: knowledge, distinction, mental processing, control, strength in practice, perspicacity, acumen, insight, comprehend and sagacity. Then, the hypothetical relationships between these components and the process of achieving wisdom were investigated. The findings showed that the hypothetical relationships between these ten components are classified into two levels of "core categories" and "active domains". Based on this, wisdom in the core has the basic elements of "cognition", " control", "diagnosis", "processing", and " strength in practice ", which led to "perception of the best interest" in the intrapersonal domain and "insight", "perception of inner motivation and goals", "perception of concepts and objects", and "acuity" in the extra personal domain. The results were the design of a process model of wisdom, which was evaluated by 6 experts in Islamic sciences and psychology, and analyzed and evaluated using (CVI).

The article delves into the pathogenesis of systemic sclerosis (SSc) with an emphasis on immunometabolism dysfunctions. SSc is a complex autoimmune connective tissue disorder with skin and organ fibrosis manifestation, vasculopathy, and immune dysregulation. A growing amount of research indicates that immunometabolism plays a significant role in the pathogenesis of autoimmune diseases, including SSc. The review explores the intricate interplay between immune dysfunction and metabolic alterations, focusing on the metabolism of glucose, lipids, amino acids, the TCA (tricarboxylic acid) cycle, and oxidative stress in SSc disease. According to recent research, there are changes in various metabolic pathways that could trigger or perpetuate the SSc disease. Glycolysis and TCA pathways play a pivotal role in SSc pathogenesis through inducing fibrosis. Dysregulated fatty acid β-oxidation (FAO) and consequent lipid metabolism result in dysregulated extracellular matrix (ECM) breakdown and fibrosis induction. The altered metabolism of amino acids can significantly be involved in SSc pathogenesis through various mechanisms. Reactive oxygen species (ROS) production has a crucial role in tissue damage in SSc patients. Indeed, immunometabolism involvement in SSc is highlighted, which offers potential therapeutic avenues. The article underscores the need for comprehensive studies to unravel the multifaceted mechanisms driving SSc pathogenesis and progression.

In this study, the insecticidal effect of the Detech® (Turkish Diatomaceous earth) DE formulation against Galleria mellonella L. was determined. The study was conducted in a laboratory environment with materials taken from beehives produced at Muş Alparslan University in 2022. Diatomaceous earth (DE) was applied in two different forms (dust and slurry DE) and as positive control GüveSavar®, which is currently used against some pests in beekeeping. DE concentrations of 0, 3, 5, and 7 g/m2 in different exposure times were tested for the control of G. mellonella larvae. As a result of all treatments, the highest mortality occurred at 7 g/m2 dust DE concentration. Larvae (3rd stage) exposed to 7 g/m2 concentration according to dust DE mortality rates reached 100% mortality after 40 hours. According to the results of the slurry DE, the larvae exposed to the slurry diatom at all concentrations achieved 100% mortality at the end of the 96 hours. When the dust and slurry DE results were examined, the direct use of dust formulations greatly accelerated the effectiveness against larvae. The study showed very promising results, suggesting that slurry DE and dust formulations could be a new alternative control method for Greater Wax Moth. In addition, for the first time, the insecticidal efficacy of DE against the honey bee pest, the greater wax moth, was determined.

Only if there is time for sports is it possible for sports to give life significance. At this point, recreational sports take center stage. Recreational activities are known to improve health, but it is assumed that this health will also make leisure time more enjoyable. Accordingly, the purpose of this relational survey research is to evaluate the link between university community members' pleasure with their leisure time and recreational well-being. The research employed an easy random sampling procedure and a descriptive scanning methodology. In the study, "Recreational Sports Well-Being Scale" and "Leisure Satisfaction Scale" were used to determine the recreational sports well-being and leisure satisfaction levels of the participants. The universe of the research is "Selçuk University Student Societies". A total of 298 people participated in the research. As 14 participants left most of the questions blank and 7 participants marked more than one variable, 21 people were excluded from the study, and 277 people, 127 women and 150 men, were included in the study. The results show that there is a substantial relationship between leisure time satisfaction and the length of time spent engaging in recreational activities and sports. On the other hand, it has been determined that the income variable is one of the most effective factors in terms of well-being and leisure satisfaction. In conclusion; leisure satisfaction and recreational sport well-being are moderately positively related. It can be stated that the gains of female participants and participants with high perceived income from recreational sports activities contribute more positively to their well-being levels.

Bu çalışma kapsamında, otomotiv sektöründe kullanılan amortisörün bağlantı ekipmanlarından biri olan klevis braketin üretiminde rol oynayan projeksiyon kaynak prosesinin iyileştirilmesi ve projeksiyon kaynak parametrelerinin klevis braketin mekanik performansını nasıl etkilediği araştırılmıştır. Bu kapsamda, 9 farklı proses kullanılarak numuneler üretilmiş ve bu numunelerin kopma testleri yapılmış ve elde edilen sonuçlara göre ANOVA analizi uygulanmıştır. Analiz sonucunda, en etkili parametrenin %80 oranında kaynak basıncı olduğu ve kaynak sıkma süresinin etkisinin ihmal edilebilecek düzeyde olduğu belirlenmiştir. Ayrıca, projeksiyon kaynağıyla yapılan 5 farklı noktanın en kritik 2 noktasının kesitlerinin makro ve mikro yapısı incelenmiştir. Kaynak süresinin artmasıyla birlikte ısıl girdinin arttığı ve bunun sonucunda mikro yapıdaki ısı tesirli alanların ve füzyon bölgesinin boyutlarının arttığı gözlemlenmiştir. Bununla birlikte, kaynak basıncının artması mikro yapıyı olumsuz yönde etkilemiştir. Ayrıca, kaynak kesitlerinin ortalama sertlik değerlerinin, bu mikro yapıdaki değişimden etkilendiği gözlenmiştir.

Addiction relapse is a complex, multifaceted and dynamic phenomenon that is influenced by biological, psychological and social factors. The purpose of this research was to analyze the lived experiences of young addicts about the role of social support in preventing addiction relapse. Husserl's descriptive phenomenological method was used in order to discover the lived experiences of young people and the role of social support in their return to addiction. The target population consisted of all young people, who were between 18 and 35 years old. They had referred to addiction treatment centers in Golestan province to get rid of addiction again. Participants were selected using purposive sampling. Sampling continued until data saturation; this goal was realized in sample 15.The data collection tool was a semi-structured interview; which lasted between 50 to 70 minutes. The data were analyzed using the 7-step Colaizzi method. In order to achieve validity and reliability of the interviews, the criteria proposed by Lincoln and Gouba. From the analysis of young people's lived experiences of the role of social support in addiction relapse, 5 main themes and 38 sub-themes were obtained. The main themes were: bad friends, insufficient family support, weak family control, family coercion and marital discord. Social support is very important in preventing addiction; And it demands special attention, especially from families.

Background: Melanosis vesicae is a rare condition characterized by the deposition of melanin within the bladder urothelium. Case presentation: We present a case of a 72-year-old male with a history of recurrent urinary retention, bladder diverticula, and concurrent Aerococcus urinary tract infection who presented with left-sided abdominal pain. Cystoscopy revealed diffuse black splotch lesions throughout the bladder and two diverticula. Histopathological examination confirmed the diagnosis of melanosis vesicae. The patient ultimately underwent an open bladder diverticulectomy. Conclusion: The potential associations between melanosis vesicae, urinary tract malignancies and concurrent conditions such as bladder diverticula and urinary infections warrant further investigation.

SARS-CoV-2, responsible for COVID-19, shares 79% and 50% of its identity with SARS-CoV-1 and MERS-CoV, respectively. It uses the same main cell attachment and entry receptor as SARS-CoV-1, which is the ACE-2 receptor. However, key residues in the receptor-binding domain of its S-protein seem to give it a stronger affinity for the receptor and a better ability to hide from the host immune system. Like SARS-CoV-1 and MERS-CoV, cytokine storms in critically ill COVID-19 patients cause ARDS, neurological pathology, multiorgan failure, and increased death. Though many issues remain, the global research effort and lessons from SARS-CoV-1 and MERS-CoV are hopeful. The emergence of novel SARS-CoV-2 variants and subvariants raised serious concerns among the scientific community amid the emergence of other viral diseases like monkeypox and Marburg virus, which are major concerns for healthcare settings worldwide. Hence, an updated review on the comparative analysis of various coronaviruses (CoVs) has been developed, which highlights the evolution of CoVs and their repercussions.

Bu çalışma kapsamında, Inconel 718 tozunun AISI 1050 imalat çeliği üzerine lazerle dolgu kaynağı işlemi uygulandı. Bu işlem sırasında, dispersiyonun mekanik özellikler üzerindeki etkisini analiz etmek için, ilgili parametrelerde üretilen numuneler üzerinde ampirik bir çalışma gerçekleştirildi. Sonuçlar, kopma gerilmesi ve süneklik için yüksek sapmalar olduğunu göstermektedir. Bu dağılım, biriken malzemede gözenekliliğin varlığından kaynaklanmaktadır. Ayrıca, en iyi sonuçlar merkez bölgedeki numunelerden alınırken, dış bölgelerden alınan numuneler üzerinde yapılan testler kopma gerilmesi ve süneklik için daha düşük değerler göstermiştir. Bunun yanında sertlik değerleri incelendiğinde ısıdan etkilenen bölgenin ana malzemeden daha sert olduğu ve bu da ısıdan etkilenen bölgenin soğuma hızından kaynaklandığı görülmüştür. Buna ilaveten de Laves fazı, zamanla kırılmaya yol açan mikro boşlukların üretilmesinin ana nedeni olduğu anlaşılmıştır.

A role for exportin 4 (XPO4) in the pathogenesis of liver fibrosis was recently identified. We sought to determine changes in hepatic XPO4 promoter methylation levels during liver fibrosis. The quantitative real-time RT-PCR technique was used to quantify the mRNA level of XPO4. Additionally, pyrosequencing was utilized to assess the promoter methylation status of XPO4. The methylation rate of the XPO4 promoter was significantly increased with fibrosis in human and mouse models, while XPO4 mRNA expression negatively correlated with methylation of its promoter. DNA methyltransferases (DNMTs) levels (enzymes that drive DNA methylation) were upregulated in patients with liver fibrosis compared to healthy controls and in hepatic stellate cells upon transforming growth factor beta (TGFβ) stimulation. The DNA methylation inhibitor 5-Aza or specific siRNAs for these DNMTs led to restoration of XPO4 expression. The process of DNA methylation plays a crucial role in the repression of XPO4 transcription in the context of liver fibrosis development.

This case report presents a unique and previously unreported case of malfunction, infection, and erosion of an inflatable penile prosthesis (IPP) resulting from iatrogenic injury during a priapism aspiration procedure performed by an emergency medicine physician. The patient, a 75-year-old male with a history of IPP placement for erectile dysfunction, presented with urinary retention and priapism, leading to inadvertent deflation of the IPP during aspiration. Subsequent evaluation revealed a pinhole opening on the scrotum, indicating infection and erosion of the prosthesis tubing. The patient underwent emergent explantation of the infected IPP, washout, cystoscopy, and insertion of a suprapubic tube. Intraoperative cultures identified Escherichia cloacae as the causative pathogen. This case highlights the importance of thorough chart review to identify patients with IPPs before aspiration procedures and emphasizes the need for healthcare provider education regarding potential complications in this patient population. Early recognition and management of such complications are crucial for optimal patient outcomes. While IPP placement remains a highly satisfactory treatment for erectile dysfunction, this case highlights the importance of vigilance to ensure the best care for patients with penile prostheses. It is noteworthy that ultimately, a new IPP was not placed in this patient due to the patient’s significant medical comorbidities.

Abstract Introduction Type 2 diabetes mellitus (T2DM) is characterized primarily by dyslipidemia and hyperglycemia due to insulin resistance. High‐density lipoprotein (HDL) play a significant role in preventing the incidence of dyslipidemia and its complications. HDL has different protective functions, such as reducing oxidation, vascular inflammation, and thrombosis; additionally, its anti‐diabetic role is one of the most significant recent discoveries about HDL and some of its constituent lipoproteins. Methods This research reviews ongoing studies and preliminary investigations into the assessment of relation between decreased level of HDL and T2DM. Results The levels of HDL and its functions contribute to glucose hemostasis and the development of T2DM through four possible mechanisms, including insulin secretion by beta cells, peripheral insulin sensitivity, non‐insulin‐dependent glucose uptake, and adipose tissue metabolic activity. Additionally, the anti‐oxidant properties of HDL protect beta cells from apoptosis caused by oxidative stress and inflammation induced by low‐density lipoprotein, which facilitate insulin secretion. Conclusion Therefore, HDL and its compositions, especially Apo A‐I, play an important role in regulating glucose metabolism, and decreased levels of HDL can be considered a risk factor for DM. Different factors, such as hypoalphalipoproteinemia that manifests as a consequence of genetic factors, such as Apo A‐I deficiency, as well as secondary causes arising from lifestyle choices and underlying medical conditions that decrease the level of HDL, could be associated with DM. Moreover, intricate connections between HDL and diabetic complications extend beyond glucose metabolism to encompass complications like cardiovascular disease and kidney disease. Therefore, the exact interactions between HDL level and DM should be evaluated in future studies.

Background: Disease risk variants are likely to affect gene expression in a context- and cell-type specific manner. The membrane bound O-acyltransferase domain containing 7 (MBOAT7) rs8736 metabolic-dysfunction-associated fatty liver disease (MAFLD)-risk variant was recently reported to be a negative regulator of toll-like receptors (TLRs) signalling in macrophages. Whether this effect is generic or cell-type specific in immune cells is unknown. Methods: We investigated the impact of modulating TLR signaling on MBOAT7 expression in peripheral blood mononuclear cells (PBMCs). We also examined whether the rs8736 polymorphism in MBOAT7 regulates this effect. Furthermore, we measured the allele-specific expression of MBOAT7 in various immune cell populations under both unstimulated and stimulated conditions. Results: We show that MBOAT7 is down-regulated by TLRs in PBMCs. This effect is modulated by the MBOAT7 rs8736 polymorphism. Additionally, we provide evidence that MBOAT7 acts primarily as a modulator of TLR signalling in mononuclear phagocytes. Conclusion: Our results highlight the importance of studying Genome-Wide Association Studies (GWAS) signals in the specific cell types in which alterations of gene expression are found.

The ever-evolving landscape of cyber threats, with ransomware at its forefront, poses significant challenges to the digital world. Windows 11 Pro, Microsoft’s latest operating system, claims to offer enhanced security features designed to tackle such threats. This paper aims to comprehensively evaluate the effectiveness of these Windows 11 Pro, built-in security measures against prevalent ransomware strains, with a particular emphasis on crypto-ransomware. Utilizing a meticulously crafted experimental environment, the research adopted a two-phased testing approach, examining both the default and a hardened configuration of Windows 11 Pro. This dual examination offered insights into the system’s inherent and potential defenses against ransomware threats. The study’s findings revealed that Windows 11 Pro does present formidable defenses. This paper not only contributes valuable insights into cybersecurity, but also furnishes practical recommendations for both technology developers and end-users in the ongoing battle against ransomware. The significance of these findings extends beyond the immediate evaluation of Windows 11 Pro, serving as a reference point for the broader discourse on enhancing digital security measures.

IntroductionDevice closure of perimembranous ventricular septal defects (pmVSD) is a successful off-label treatment alternative. We aim to report and compare the outcomes of pmVSD closure in children weighing less than 10 kg using Amplatzer Duct Occluder II (ADOII) and Konar-MF VSD Occluder (MFO) devices.MethodsRetrospective clinical data review of 52 children with hemodynamically significant pmVSD, and sent for transcatheter closure using ADOII and MFO, between January 2018 and January 2023. Baseline, procedural, and follow-up data were compared according to the implanted deviceResultsADOII devices were implanted in 22 children with a median age of 11 months (IQR, 4.1–14.7) and weight of 7.4 kg (IQR, 2.7–9.7). MFO devices were implanted in 30 children with a median age of 11 months (IQR, 4.8–16.6) and weight of 8 kg (IQR, 4.1–9.6). ADOII were implanted (retrograde, 68.1%) in defects with a median left ventricular diameter of 4.6 mm (IQR, 3.8–5.7) and right ventricular diameter of 3.5 mm (IQR, 3.1–4.9) while MFO were implanted (antegrade, 63.3%) in defects with a median left ventricular diameter of 7 mm (IQR, 5.2–11.3) (p > 0.05) and right ventricular diameter of 5 mm (IQR, 2.0, 3.5–6.2) (p

Background: Heart rate variability (HRV) is a sign of the cardiac autonomic nervous system. Its evaluation in pediatric ventricular septal defect (VSD) cases before and after transcatheter closure contributes to an understanding of cardiac autonomic control. Methods: Nineteen children with VSDs treated with transcatheter closure and 18 healthy children were enrolled in this study. A 24-h Holter rhythm monitor was applied to all patients before VSD closure and to those in the control group. Holter rhythm monitoring was repeated at three months in the patient group. HRV parameters were measured using the Cardio Scan Premier 12® program. Frequency-domain (total power; very-low-frequency, low-frequency (LF), and high-frequency (HF) indices; and the LF/HF ratio) and time-domain (standard deviation of all RR intervals (SDNN), standard deviation of 5-min averages of RR intervals (SDANN), the SDNN index, percentage of the difference between adjacent RR intervals, and the square root of the mean of the sum of square differences between adjacent filtered RR intervals) parameters were assessed. Results: Before the procedure, SDNN, SDANN, and total power values were lower in the patient group than in the control group; other parameters were similar in the two groups. No significant difference in the SDNN, SDANN, or total power was detected between the patient and control groups in the third month, indicating that autonomic control of patients’ hearts became normal during the third postoperative month. No correlation was detected between any hemodynamic parameters and any time-domain or frequency-domain parameters before closure. Conclusion: This study showed that transcatheter closure of VSDs changed HRV parameters in pediatric patients. Resumo: Introdução: A variabilidade da frequência cardíaca (VFC) é uma manifestação do sistema nervoso autónomo cardíaco. A sua avaliação em casos pediátricos de comunicação interventricular (CIV) antes e após o encerramento percutâneo contribui para a compreensão do controlo autonómico cardíaco. Métodos: Neste estudo foram incluídas 19 crianças com CIV tratadas com encerramento percutâneo e 18 crianças saudáveis. Foi utilizado um ECG de Holter de 24 horas em todos os doentes antes do encerramento da CIV e no grupo de controlo. A monitoração com ECG de Holter foi repetida aos três meses no grupo de doentes. Os parâmetros de VFC foram medidos utilizando o programa Cardio Scan Premier 12®. Domínio de frequência [potência total; índices de frequência muito baixa, de frequência baixa (FB), de frequência alta (FA) e relação FB/FA]. Domínio temporal [desvio-padrão de todos os intervalos RR (SDNN), desvio-padrão de médias de 5 minutos de intervalos RR (SDANN), índice SDNN, percentagem da diferença entre intervalos RR adjacentes e a raiz quadrada da média da soma das diferenças quadradas entre intervalos RR filtrados adjacentes]. Resultados: Antes do procedimento, os valores SDNN, SDANN e da potência total foram mais baixos no grupo de doentes do que no grupo controlo; os outros parâmetros foram semelhantes nos dois grupos. Não foi detetada qualquer diferença significativa nos valores de SDNN, SDANN ou na potência total entre os doentes e o grupo controlo ao terceiro mês, indicando que o controlo autónomo dos corações dos doentes se tornou normal no pós-operatório. Não foi detetada qualquer correlação entre qualquer parâmetro hemodinâmico e qualquer parâmetro de domínio temporal ou de domínio de frequência antes do encerramento. Conclusão: Este estudo demonstrou que o encerramento percutâneo da CIV alterou os parâmetros da VFC em doentes pediátricos.

Devices may be used for special purposes different than their production purpose. For instance, Amplatzer Ductal Occluder is actually designed for duct closure and its usage for closing defects other than ductus is named as off-label. The aim of this study is to emphasize off-label use of device: not only for PDA and VSD but also for other various defects. This study is designed retrospectively, performed by the evaluation catheterization records of patients in whom ADO II and ADO II-AS devices were used in Erciyes University Medical Faculty Children Hospital, Pediatric Cardiology Department between 2011 and 2018. Patients’ demographic criteria: age, weight at the time of procedure was gathered. The diagnosis, size of device, follow-up period and complications were also noted. From April 2011 to March 2018, a total of 121 patients underwent transcatheter closure by ADO II and 66 patients by ADO II AS. The number of PDA closure with ADO II was 48; with ADO II AS were 62. Rest of the procedures were all off-label. Types of off-label procedures performed were: VSD closure, residual mitral cleft closure, Aorta-Right atrium tunnel closure, pulmonary arteriovenous fistula occlusion, aorta-pulmonary window closure, and occlusion of the artery feeding accessory lobe in scimitar syndrome, Gerbode defect occlusion. Up to our knowledge; this study includes the largest pediatric case series with various different congenital heart defects which were closed with ADO-II. Also our ADO-II occluded VSD case series is one of the largest series in the literature with almost 6 years’ follow-up. We believe in that ADO-II device may be an alternative in percutaneous closure of various rare heart defects. It is used successfully for non-ductal defects with low complication and high compliance rates.

Abstract Background Total knee arthroplasty (TKA) is an effective procedure for patients with a variety of knee conditions. The main cause of aseptic TKA failure is implant loosening, which has been linked to poor cement mantle quality. Cementless components were introduced to offer better longer-term biological fixation through osseointegration; however, early designs led to increased rate of revision due to a lack of initial press-fit and bony ingrowth. Newer highly porous metal designs may alleviate this issue but randomised data of fully uncemented TKA (tibial, femoral, patella) is lacking. The aim of the Knee-Fix study is to investigate the long-term implant survival and patient outcomes of fully uncemented compared with cemented fixation in TKA. Our study hypothesis was that uncemented TKA would be as clinically reliable and durable as the gold-standard cemented TKA. Methods The Knee-Fix study is a two-arm, single-blinded, non-inferiority randomised controlled trial with 160 patients in each arm and follow-up at 6 weeks, 6 months, 12 months, 24 months, 5 years and 10 years. The primary outcome of interest is implant fixation, which will be measured by assessment of postoperative progressive radiolucencies with the Knee Society Total Knee Arthroplasty Roentgenographic Evaluation and Scoring System. Secondary outcome measures are patient-reported outcomes, measured using Oxford Knee Score (OKS), International Knee Society System (IKSS), Forgotten Joint Score-12 (FJS-12), EuroQol (EQ-5D-5L), VAS Pain, Patient Satisfaction Score and Net Promoter Score. Discussion While cemented fixation remains the gold standard, a growing proportion of TKA are now implanted cementless. Highly porous metal cementless components for TKA can offer several benefits including potentially improved biological fixation; however, long-term outcomes need further investigation. This prospective study will help discern long-term differences between the two techniques. Trial registration Australian New Zealand Clinical Trials Registry ACTRN12616001624471 . Registered trial name: Knee-Fix study (Cemented vs Uncemented Total Knee Replacement). Registered on 24 November 2016.

Hyperactivation of the toll-like receptors (TLRs) have been implicated as risk factors for more severe forms of disease in COVID-19 and metabolic (dysfunction) associated fatty liver disease (MAFLD). Here the authors report that MBOAT7 is reduced in macrophages of patients with MAFLD and COVID-19, and acts as a negative regulator of TLR signalling.

The present study tried to achieve a conceptual model of wisdom and examine its validating through three methods of linguistic semantics, grounded theory, and survey descriptive method. By reviewing the concept of wisdom in the fields of vocabulary and psychology, 23 primary concepts related to wisdom were identified in Islamic sources. After analyzing content validity, nineteen concepts were left out of 23 concepts. In the phase of collecting the documents, there were 2149 data resulted from seeking the data related to nineteen concepts which were screened in the two stages of book weakness and the lack of conceptual relevance. It resulted in omitting 1039 documents and leaving 1110 Islamic data as well as twelve concepts for modeling. These concepts, including reason (Aghl), intellect (Hekmat), distinction (Tamiz), knowledge (Marefat), science (Elm), mental processing (Pardazesh Zehni), understanding (Fahm), insight (Basirat), acumen (Ferasat), perspicacity (Kiasat), Fiqh, ingenuity (Fetanat), and sagacity (Zekavat), were analyzed using grounded theory in the four stages. By the judgmental sampling, 1276 primary open codes, 344 centralized open codes, 84 axial codes, and 19 core categories were identified. The proportionality among the core categories and comparing them with the documents showed that Islamic wisdom consists of ten conceptual elements, including knowledge (Danesh), distinction (Tamiz), mental processing (Pardazesh Zehni), control (Mahar), certainty in action (Etghan dar Amal), perspicacity (Kiasat), acumen (Ferasat), insight (Basirat), Fiqh, and cleverness (Tizfahmi). These elements were analyzed by eleven experts and Content Validity Index (CVI). In addition, the experts confirmed the validity of elements.