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Erdheim-Chester disease (ECD) is a rare, multisystemic, inflammatory, non-Langerhans cell histiocytic neoplasm. The discovery of recurrent and somatic mutations in the mitogen-activated protein kinase signaling pathway, most commonly BRAFV600E, has led to a reclassification of ECD from an inflammatory disorder to a neoplastic process. It is now included in the revised 2016 World Health Organization classification of hematopoietic tumors and in the Langerhans group in the revised 2016 Histiocytosis Classification of the Histiocyte Society. When symptomatic, ECD most commonly manifests with bone pain and fatigue. Also, neurologic manifestations, central diabetes insipidus, exophthalmos, and periorbital xanthelasma-like lesions are frequently encountered. Pathologic findings may vary depending on the site of biopsy and may display a spectrum of features. Thus, due to the diverse clinical presentation and variable histologic findings, imaging can often show the first sign of the disease. Radiologic findings are, however, interpreted in conjunction with clinical and histologic findings to establish the diagnosis of ECD. From providing classic findings that facilitate diagnosis to helping radiologists determine the extent of disease and predicting a prognosis, the role of radiology in ECD has evolved with the understanding of the disease itself. Insights into the molecular pathogenesis and the development of targeted therapeutic agents along with approval of vemurafenib and cobimetinib have necessitated revision of the guidelines for the management of ECD. The authors discuss various radiologic findings of ECD and differential diagnoses by using an organ system-based approach and briefly describe the revised consensus recommendations for evaluation, diagnosis, and treatment based on the International Medical Symposia on ECD from a radiologist's perspective. ©RSNA, 2024 Supplemental material is available for this article. The full digital presentation is available online.
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Doença de Erdheim-Chester , Doença de Erdheim-Chester/diagnóstico por imagem , Humanos , Diagnóstico DiferencialRESUMO
CD44+ cancer stem cells (CSCs) are believed to account for drug resistance and tumour recurrence due to their potential to self-renew and differentiate into heterogeneous lineages. Therefore, efficient treatment strategies targeting and eliminating these CSCs are required. The flavonolignan, Silibinin, has gained immense attention in targeting CD44+ CSCs as it alters functional properties like cell cycle arrest, apoptosis, inhibition of invasion and metastasis and also inhibits a range of molecular pathways. However, its limited bioavailability is a major hurdle in asserting Silibinin as a translational therapeutic agent. Combinatorial therapy of Silibinin with conventional chemotherapeutic drugs is an alternative approach in targeting CD44+ CSCs as it increases the efficacy and reduces the cytotoxicity of chemotherapeutic drugs, thus preventing drug resistance. Certain Silibinin-conjugated nano-formulations have also been successfully developed, through which there is improved absorptivity/bioavailability of Silibinin and a decrease in the concentration of therapeutic drugs leading to reduced cytotoxicity. In this review, we summarise the effectiveness of the synergistic therapeutic approach for Silibinin in targeting the molecular mechanisms of CD44+ CSCs and emphasise the potential role of Silibinin as a novel therapeutic agent.
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Neoplasias , Humanos , Receptores de Hialuronatos/metabolismo , Receptores de Hialuronatos/uso terapêutico , Neoplasias/tratamento farmacológico , Células-Tronco Neoplásicas , Silibina/farmacologiaRESUMO
Dermatomyositis is a heterogeneous idiopathic inflammatory myopathy associated with various cutaneous manifestations and variable presence of myositis, interstitial lung disease, and other visceral organ involvement. An accurate diagnosis of dermatomyositis requires correlating clinical examination findings with serological and histological findings. Familiarity with pathognomonic and common cutaneous manifestations of dermatomyositis, which are highlighted here, can be especially helpful in making an accurate diagnosis. Additionally, evaluating patients for presence of myositis-specific autoantibodies can further support or refute a dermatomyositis diagnosis. When present, myositis-specific autoantibodies can also help guide workups for various dermatomyositis-associated manifestations, as each is associated with relatively distinct clinical characteristics. Evaluating patients for various systemic manifestations often relies on expert opinion recommendations; however, societal guideline statements concerning the evaluation of some manifestations have recently been described. Although malignancy-associated dermatomyositis is a well-accepted subtype, there is limited evidence to support extensive malignancy screening has a favorable benefit-risk ratio in most dermatomyositis patients. However, recent research has uncovered novel associations between dermatomyositis and malignancy, suggesting the possibility of identifying high-risk subsets of dermatomyositis patients in whom malignancy screening may have a high value. Treatment for dermatomyositis has remained largely unchanged over the past several decades. Although many dermatomyositis patients can be effectively treated with current options, either as monotherapy or with combination regimens, there is a need for more targeted and effective DM therapies, in general, and for MDA5(+) dermatomyositis-associated rapidly progressive interstitial lung disease. Fortunately, significant current and emerging research activities evaluating various novel medications for dermatomyositis provide hope for exciting future advances in patients with this intriguing immune-mediated disease.
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BACKGROUND: The role of spirituality in people's lives, particularly the lives of health professionals, as well as its impact on mental health issues like anger and aggressiveness and resilience, are particularly significant. Therefore, the purpose of this study was to examine the link between the propensity for violence and personal resilience in dental students by taking into account the function of spirituality as a mediator. MATERIALS AND METHODS: In this cross-sectional questionnaire survey, 211 volunteer dentistry students participated from a government institute in Jammu and Kashmir, India using a stratified sample procedure. The Spiritual Orientation Scale, Buss and Perry Aggression Questionnaire, and Connor-Davidson Resilience Scale were used to gather the data. SPSS version 20.0 was used for conducting a bivariate analysis to ascertain the directionality connection between the research variables. In addition; structural equation modelling analysis was conducted by Smart PLS. RESULTS: According to the findings, resilience and spirituality have a substantial and positive link (r = 0.468 r = 0.023). Aggressiveness and resilience revealed a statistically significant inverse link (r = 0.325, P = 0.04), but aggression and spirituality had no significant correlation. Spirituality played a substantial mediation influence in the indirect pathway of violence on resilience (P = 0.001). CONCLUSION: According to the study's findings, spirituality can help pupils become more resilient as individuals and can act as a helpful intermediary between aggressiveness and resilience.
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While a plethora of articles discuss management of deep venous thromboses in extremities, there is a relative scarcity of literature comprehensively describing intra-abdominal venous thromboses, and their management. Intra-abdominal venous thromboses include iliocaval venous obstruction (ICVO), hepatic venous thrombosis (HVT), portal venous thrombosis (PVT), renal vein thrombosis (RVT), splenic vein thrombosis (SVT), and gonadal vein thrombosis (GVT); each of which provides unique microenvironmental challenges to management. Doppler ultrasound is the first line imaging modality for diagnosis, and computed tomography and magnetic resonance imaging can help define the extent of thrombus burden and aid with interventional planning. Systemic anticoagulation remains the common medical treatment for intra-abdominal venous thrombosis, however, catheter directed thrombolysis and thrombectomy show positive outcomes in ICVO, HVT, PVT, and RVT, with transjugular intrahepatic portosystemic shunt (TIPS) creation especially beneficial in HVT and PVT. In this review article, we describe pathophysiology, clinical features, imaging findings, and current management options for intra-abdominal venous thromboses.
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INTRODUCTION: Endodontic therapy requires meticulous root canal debridement, pathogen elimination, and effective obturation to prevent microbial intrusion. The presence of the smear layer hinders sealer penetration, compromising sealing effectiveness. Sodium hypochlorite and chlorhexidine are esteemed endodontic irrigants. Herbal extracts like neem and tulsi, with antimicrobial and anti-inflammatory properties, show promise for root canal irrigation. The study aimed to evaluate the efficacy of various irrigants in removing the smear layer and enhancing push-out bond strength at different root canal levels. MATERIALS AND METHODS: One hundred mandibular premolars with single canals were collected, and 50 samples each were used for the smear layer and push-out bond strength analysis. Neem and tulsi extracts were prepared for irrigation. Teeth were decoronated, and up to 30 (6%) canals were prepared and were randomly divided into five groups based on irrigants used. A smear layer examination was conducted after longitudinally sectioning the tooth and sections were observed in a scanning electron microscope (SEM). Obturation was done in the remaining samples, and the push-out bond strength was assessed using a universal test machine. RESULTS: Sodium hypochlorite showed the highest smear layer removal efficacy followed by chlorhexidine, neem, tulsi leaves with rose water extract, and normal saline. Chlorhexidine exhibited the highest push-out bond strength, with the coronal third presenting the strongest values, followed by neem, tulsi with rose water, normal saline, and sodium hypochlorite. CONCLUSION: The study underscores the potential of herbal irrigants in endodontic therapy, indicating promising results while emphasizing the necessity for further clinical trials to validate their efficacy and other properties.
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BACKGROUND: Inflammatory bowel disease (IBD)-associated peripheral spondyloarthritis (pSpA) decreases quality of life and remains poorly understood. Given the prevalence of this condition and its negative impact, it is surprising that evidence-based disease definitions and diagnostic strategies are lacking. This systematic review summarizes available data to facilitate development and validation of diagnostics, patient-reported outcomes, and imaging indices specific to this condition. METHODS: A literature search was conducted. Consensus or classification criteria, case series, cross-sectional studies, cohort studies, and randomized controlled trials related to diagnosis were included. RESULTS: A total of 44 studies reporting data on approximately 1500 patients with pSpA were eligible for analysis. Data quality across studies was only graded as fair to good. Due to large heterogeneity, meta-analysis was not possible. The majority of studies incorporated patient-reported outcomes and a physical examination. A total of 13 studies proposed or validated screening tools, consensus, classification, or consensus criteria. A total of 28 studies assessed the role of laboratory tests, none of which were considered sufficiently accurate for use in diagnosis. A total of 17 studies assessed the role of imaging, with the available literature insufficient to fully endorse any imaging modality as a robust diagnostic tool. CONCLUSIONS: This review highlights existing inconsistency and lack of a clear diagnostic approach for IBD-associated pSpA. Given the absence of an evidence-based approach, a combination of existing criteria and physician assessment should be utilized. To address this issue comprehensively, our future efforts will be directed toward pursuit of a multidisciplinary approach aimed at standardizing evaluation and diagnosis of IBD-associated pSpA.
This systematic review highlights the lack of an evidence-based approach to the diagnosis of inflammatory bowel diseaseassociated peripheral spondyloarthritis and the need to standardize evaluation and diagnosis via multidisciplinary collaboration with development of patient-reported outcomes and imaging indices.