Publications by authors named "Lengyel A"

The combinations of BRAF inhibitor-based targeted therapies with immune checkpoint inhibitors currently represent less common therapeutic approaches in advanced melanoma. The aim of this study was to assess the safety and efficacy of currently available melanoma treatments by conducting a systematic review and network meta-analysis. Four databases were systematically searched for randomized clinical studies that included patients with advanced/metastatic melanoma receiving chemotherapy, immune checkpoint inhibitors, BRAF/MEK inhibitor therapy, or combinations thereof.

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To assess the suitability of genome sequencing (GS) as the second step in the diagnostics of patients with the features of 11p15.5-associated imprinting disorders (ImpDis: Silver-Russell syndrome [SRS], Beckwith-Wiedemann syndrome [BWS]), we performed short-read GS in patients negatively tested for imprinting disturbances. Obtaining a genetic diagnosis for patients with the features of these syndromes is challenging due to the clinical and molecular heterogeneity and overlap, and many patients remain undiagnosed after the currently suggested stepwise diagnostic workup.

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Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.

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: Understanding movement development in children with autism spectrum disorder (ASD) is critical for creating effective intervention strategies. This study aims to explore parental perspectives on the movement development of children with ASD, focusing on identifying common challenges and successful strategies. The objective is to analyze the experiences of parents to highlight the most effective approaches to support motor, communication, and social skills development in these children.

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Article Synopsis
  • MITF (Microphthalmia-associated transcription factor) is crucial for melanocyte function and is linked to melanoma development, helping cancer cells survive therapy by regulating antioxidant responses.
  • It promotes antioxidant programs that protect melanoma cells from damage caused by reactive oxygen species (ROS), with a clear association between MITF levels and antioxidant defenses in cell lines and patient samples.
  • Experimental studies, including a zebrafish melanoma model, confirm that MITF reduces ROS-related DNA damage through direct regulation of specific target genes, establishing its role in enhancing cellular antioxidant capacity.
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Pediatric forearm fractures represent a substantial proportion of childhood injuries, requiring effective and minimally invasive treatments. Our study investigated the mid-term outcomes of biodegradable poly-L-lactide-co-glycolide (PLGA) intramedullary implants in managing diaphyseal forearm fractures in children. A follow-up cohort study was conducted with 38 patients treated with PLGA implants.

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Introduction: While Immune checkpoint inhibition (ICI) therapy shows significant efficacy in metastatic melanoma, only about 50% respond, lacking reliable predictive methods. We introduce a panel of six proteins aimed at predicting response to ICI therapy.

Methods: Evaluating previously reported proteins in two untreated melanoma cohorts, we used a published predictive model (EaSIeR score) to identify potential proteins distinguishing responders and non-responders.

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Background And Purpose:

Human brain aneurysms may often prove fatal if not re­cognized in time and treated accordingly. The understanding of development and rupture of aneurysms can significantly be improved by the application of numerical modelling, which in turn, requires the knowledge of mechanical properties of vessel wall. This study aims to identify assumed differences with respect to age, sex, spatial orientation, and rupture by utilizing detailed statistical analysis of uniaxial tensile measurements of human brain aneurysm samples, performed by the authors in a previous project.

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Background: Cardiovascular failure is recognized as a common final pathway at the end of life but there is a paucity of data describing terminal arrhythmias.

Aim: We aimed to describe arrhythmias recorded peri-mortem in critically ill patients.

Study Design: We enrolled intensive care unit patients admitted to two tertiary Canadian medico-surgical centres.

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While Immune checkpoint inhibition (ICI) therapy shows significant efficacy in metastatic melanoma, only about 50% respond, lacking reliable predictive methods. We introduce a panel of six proteins aimed at predicting response to ICI therapy. Evaluating previously reported proteins in two untreated melanoma cohorts, we used a published predictive model (EaSIeR score) to identify potential proteins distinguishing responders and non-responders.

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Immune-mediated inflammatory disease (IMID) patients including psoriasis, inflammatory arthritides and bowel diseases have a higher risk of developing cardiovascular (CV) diseases compared to the general population. The increased CV risk may be promoted by tumour necrosis factor (TNF)-α-mediated immunological processes, which are present both in the pathomechanism of IMIDs and atherosclerosis. Our objective was to comprehensively investigate the effect of TNF inhibitors (TNFi) on CV risk compared with conventional therapies in IMIDs.

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Hyperelastic constitutive laws in biomechanics are used to model soft tissues, and material model parameters are often determined by performing curve fitting on data from uniaxial or biaxial tensile tests. The strain energy function of the applied constitutive law must to be energetically stable; however, this condition is not inherently provided by most currently available models. This study provides a procedure to determine stable strain energy functions in a biaxial strain space based on either uniaxial or biaxial tensile tests.

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We have previously observed phenotypic and developmental changes upon the ectopic expression of the RUNX3 or the ZBTB46 transcription factors in mouse embryonic stem cell (ESC) derived progenitors. In this study, we evaluated the gene expression profiles of the RUNX3- and the ZBTB46-instructed murine ESCs with RNA-seq testing two next-generation sequencing technologies. We compared the DNA nanoball-based DNBSEQ G400 sequencer (MGI) with the bridge-PCR-based NextSeq 500 instrument (Illumina) for RNA sequencing.

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Unlabelled: Microphthalmia-associated transcription factor (MITF) plays pivotal roles in melanocyte development, function, and melanoma pathogenesis. MITF amplification occurs in melanoma and has been associated with resistance to targeted therapies. Here, we show that MITF regulates a global antioxidant program that increases survival of melanoma cell lines by protecting the cells from reactive oxygen species (ROS)-induced damage.

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The existing plant trait databases' applicability is limited for studies dealing with the flora and vegetation of the eastern and central part of Europe and for large-scale comparisons across regions, mostly because their geographical data coverage is limited and they incorporate records from several different sources, often from regions with markedly different climatic conditions. These problems motivated the compilation of a regional dataset for the flora of the Pannonian region (Eastern Central Europe). PADAPT, the Pannonian Dataset of Plant Traits relies on regional data sources and collates data on 54 traits and attributes of the plant species of the Pannonian region.

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Background: Atrial fibrillation (AF) is often detected for the first time in patients who are hospitalized for another reason. Long-term risks for AF recurrence in these patients are unclear.

Objective: To estimate risk for AF recurrence in patients with new-onset AF during a hospitalization for noncardiac surgery or medical illness compared with a matched population without AF.

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Fabry disease (FD) is a multisystemic X-linked lysosomal storage disease that presents with angiokeratomas (AKs). Our objective was to investigate the clinical and morphologic features of AKs and to present two experimental techniques, multispectral imaging (MSI) and non-linear microscopy (NLM). A thorough dermatological examination was carried out in our 26 FD patients and dermoscopic images ( = 136) were evaluated for specific structures.

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NANS-CDG is a congenital disorder of glycosylation (CDG) caused by biallelic variants in NANS, encoding an essential enzyme in de novo sialic acid synthesis. It presents with intellectual developmental disorder (IDD), skeletal dysplasia, neurologic impairment, and gastrointestinal dysfunction. Some patients suffer progressive intellectual neurologic deterioration (PIND), emphasizing the need for a therapy.

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Aims: Electrical cardioversion is commonly used to restore sinus rhythm in patients with atrial fibrillation (AF), but procedural technique and clinical success vary. We sought to identify techniques associated with electrical cardioversion success for AF patients.

Methods And Results: We searched MEDLINE, EMBASE, CENTRAL, and the grey literature from inception to October 2022.

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Background: Neurodevelopmental disorders are genetically heterogeneous pediatric conditions. The first tier diagnostic method for uncovering copy number variations (CNVs), one of the most common genetic etiologies in affected individuals, is chromosomal microarray (CMA). However, this methodology is not yet a routine molecular cytogenetic test in many parts of the world, including Hungary.

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The strengthening of timber beams with carbon-fibre-reinforced polymers (CFRP) has been widely used in the last decades to enhance the behaviour of historical or new timber structures, usually for bending. While considerable improvement in capacity and ductility is typically achieved, the increase in stiffness was, in many cases, well short of analytical expectations, which tend to overestimate stiffness. This study addresses the problem by investigating the underlying mechanical behaviour using experimental, analytical, and numerical tools on a sample of Norway spruce () beams reinforced with carbon-fibre fabric.

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The application of fibre-reinforced polymers (FRP) for strengthening timber structures has proven its efficiency in enhancing load-bearing capacity and, in some cases, the stiffness of structural elements, thus providing cost-effective and competitive alternatives both in new design and retrofitting existing historical buildings. Over the last few decades, several reinforcing materials and techniques evolved, and considerable progress was made in numerical modelling, especially using the finite element method. As this field of research has become extensive and diversified, as well as numerous contradicting results have emerged, a thorough review is necessary.

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Article Synopsis
  • - The study aimed to evaluate how different anti-arrhythmic drugs (AADs) affect the restoration and maintenance of sinus rhythm in patients with atrial fibrillation (AF) undergoing electrical cardioversion.
  • - A systematic review and network meta-analysis of 28 randomized controlled trials found that Class III AADs and amiodarone significantly improved the acute restoration of sinus rhythm compared to no treatment or rate control.
  • - Amiodarone was shown to be the most effective in maintaining sinus rhythm long-term after electrical cardioversion, while Class Ic and Class III AADs also proved effective, albeit to a lesser extent.
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Background: Silver-Russell syndrome (SRS) is a genetic disorder characterized by intrauterine and postnatal growth restriction, relative macrocephaly at birth, body asymmetry and typical facial features. Clinical and molecular heterogeneity is described in SRS. Common causes are loss of methylation of the imprinting center 1 in 11p15 and maternal uniparental disomy of chromosome 7.

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Purpose: The purpose of this randomized trial was to compare the outcomes of using a 3-point prefabricated orthosis with elastic tape versus cast immobilization for the management of nonsurgical mallet finger.

Methods: This study was conducted in a single center. Individuals with a mallet injury requiring nonsurgical management were randomized to 6 weeks of full-time immobilization with either a 3-point prefabricated orthosis and elastic tape or a cast for distal interphalangeal joint extension.

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