bluuebunny/crossref_metadata_2025_split huggingface dataset:
Severe ACTA1-related nemaline myopathy: intranuclear rods, cytoplasmic bodies, and enlarged perinuclear space as characteristic pathological features on muscle biopsies
Authors: Labasse Clémence , Brochier Guy , Taratuto Ana-Lia , Cadot Bruno , Rendu John , Monges Soledad , Biancalana Valérie , Quijano-Roy Susana , Bui Mai Thao , Chanut Anaïs , Madelaine Angéline , Lacène Emmanuelle , Beuvin Maud , Amthor Helge , Servais Laurent , de Feraudy Yvan , Erro Marcela , Saccoliti Maria , Neto Osorio Abath , Fauré Julien , Lannes Béatrice , Laugel Vincent , Coppens Sandra , Lubieniecki Fabiana , Bello Ana Buj , Laing Nigel , Evangelista Teresinha , Laporte Jocelyn , Böhm Johann , Romero Norma B. 
Published: July 2022

AbstractNemaline myopathy (NM) is a muscle disorder with broad clinical and genetic heterogeneity. The clinical presentation of affected individuals ranges from severe perinatal muscle weakness to milder childhood-onset forms, and the disease course and prognosis depends on the gene and mutation type. To date, 14 causative genes have been identified, and ACTA1 accounts for more than half of the severe NM cases. ACTA1 encodes α-actin, one of the principal components of the contractile units in skeletal muscle. We established a homogenous cohort of ten unreported families with severe NM, and we provide clinical, genetic, histological, and ultrastructural data. The patients manifested antenatal or neonatal muscle weakness requiring permanent respiratory assistance, and most deceased within the first months of life. DNA sequencing identified known or novel ACTA1 mutations in all. Morphological analyses of the muscle biopsy specimens showed characteristic features of NM histopathology including cytoplasmic and intranuclear rods, cytoplasmic bodies, and major myofibrillar disorganization. We also detected structural anomalies of the perinuclear space, emphasizing a physiological contribution of skeletal muscle α-actin to nuclear shape. In-depth investigations of the nuclei confirmed an abnormal localization of lamin A/C, Nesprin-1, and Nesprin-2, forming the main constituents of the nuclear lamina and the LINC complex and ensuring nuclear envelope integrity. To validate the relevance of our findings, we examined muscle samples from three previously reported ACTA1 cases, and we identified the same set of structural aberrations. Moreover, we measured an increased expression of cardiac α-actin in the muscle samples from the patients with longer lifespan, indicating a potential compensatory effect. Overall, this study expands the genetic and morphological spectrum of severe ACTA1-related nemaline myopathy, improves molecular diagnosis, highlights the enlargement of the perinuclear space as an ultrastructural hallmark, and indicates a potential genotype/phenotype correlation.
Analytical studies on commercial artists’ colour charts from Das Deutsche Farbenbuch (1925)—identification of synthetic and natural organic colourants by Raman microscopy, surface-enhanced Raman spectroscopy and metal underlayer ATR-FTIR spectroscopy
Authors: Sessa Clarimma , Steuer Christoph , Quintero Balbas Diego , Sciutto Giorgia , Prati Silvia , Stege Heike 
Published: July 2022

Abstract
Historical colour charts provide a rich and often well-dated reference materials source for studying the chemical composition of all kinds of commercial brands of artists’ paints. This article presents the results of an extensive analytical study of more than 80 paint hues from 11 colour charts that are included in the German standard book Das Deutsche Farbenbuch by H. Trillich (1925, Part II). Our research focused on the identification of synthetic organic pigments, whose quickly increasing significance for artists’ paints in the early twentieth century is impossible to evaluate by documentary source research alone. A stepwise procedure combining different non- or minimally invasive vibrational spectroscopy techniques—Normal Raman and Surface-Enhanced Raman spectroscopy as well as Metal Underlayer Attenuated Total Reflection Fourier-transform Infrared Spectroscopy—allowed the identification of 18 different organic colourants in artists’ watercolours, tempera and oil colours from six German manufacturers. In addition, micro-X-Ray Fluorescence spectroscopy was applied to determine the elemental pattern of substrates, fillers, and admixed inorganic pigments. In addition to a few traditional natural organic colourants (dark and rose madder lake, cochineal lake), most of the identified compounds comprised synthetic organic pigments or synthetic dyes from various chemical classes (indigo, anthraquinone, monoazo, ß-naphthol, xanthene, triarylcarbonium, nitroso, and azine compounds). Some of these have not or only rarely been reported in artists’ paints so far. Since the identified organic colourants have mainly poor to fair (only sometimes good) fastness to light according to modern standards and partially also to solvents typically used in conservation treatments, it is evident that works of art from this period should be treated keeping in mind the possible presence of such colourants, when planning both interventive treatments and preventive measures.
Detecting multiscale periodicity from the secular effusive activity at Santiaguito lava dome complex (Guatemala)
Authors: Massaro Silvia , Costa Antonio , Sulpizio Roberto , Coppola Diego , Soloviev Anatoly 
Published: July 2022

AbstractSantiaguito, Guatemala, represents one of the best cases of active lava dome complex in the world, producing lava flow effusion, weak explosive activity, and cycles of lava dome extrusion over varying timescales. Since the inception in 1922, it has shown a remarkable constant eruptive activity, characterized by effusion of blocky domes and lava flows punctuated by moderate explosions of gas-and-ash and pyroclastic flows. In this study, we reconstruct the time evolution of discharge rates of Santiaguito across one entire century, from 1922 to 2021, combining, for the more recent activity, new satellite thermal data. By using discrete Fourier transform (DFT) and Morlet wavelet analyses, we identify three fundamental periodicities in subsets of the 1922–2021 time-series: (i) long term (ca. 10 years), (ii) intermediate term (ca. 3.5 years), and (iii) short term (from ca. 1 year to ca. 3 months), which are comparable with those observed at other lava dome eruptions at calc-alkaline volcanoes. Such inferred periodicities provide a powerful tool for the interpretation of the non-linear eruptive behaviour and represent a pivotal benchmark for numerical modelling aimed to reconstruct the dynamics of the magma feeding system based on a time-averaged discharge rate dataset.
Graphical Abstract
Developing an experimental model of early knee osteoarthritis after medial meniscus posterior root release: an in vivo study
Authors: Dzidzishvili Lika , López-Torres Irene Isabel , Guerrero Carlos Carnero , Calvo Emilio 
Published: July 2022

Abstract
Purpose
To develop a predictable and reproducible model of knee osteoarthritis after medial meniscus posterior root release.

Methods
Posteromedial meniscal root tears were created in 12 White New Zealand rabbit knees. The contralateral limbs were used as healthy controls. The animals were euthanized at 16 weeks postoperatively; tissue samples of femoral and tibial articular cartilage were collected and processed for macro and microscopic analyses to detect signs of early degeneration. Clinical evaluation of the weight-bearing status on the affected knee was conducted at 0-, 4-, 8-, and 16-weeks postoperatively.

Results
Early and severe osteoarthritic changes were the hallmark and the main findings after 16-weeks post-surgery. Macroscopically, extensive osteoarthritic changes were observed across the femoral condyle and tibial plateau. Microscopic finding included ulcerations, fissures, fibrillations, pitting, and loss of the superficial layer. Cellularity was diminished, the normal pattern of distribution in columns was lost, and subchondral bone exposure was also evident.

Conclusions
This study describes a novel model of knee osteoarthritis that may guide the development of tailored interventions to delay or prevent knee osteoarthritis. This knowledge could shift the current treatment paradigm toward more conservative and knee salvageable treatment options and increase surgeons’ awareness of this injury pattern. Such considerations may have a positive impact on clinical decision-making and subsequent patient-reported clinical outcomes.

Design
Controlled laboratory study.

Level of evidence
II.
Mesenteric desmoid fibromatosis entrapping metastatic urothelial carcinoma: a unique collision tumor or fibromatosis-like variant?
Authors: Gitto Lorenzo , Vandermeer Thomas , Lubin David J. , Zaccarini Daniel J. 
Published: July 2022

AbstractA collision tumor is a neoplastic lesion comprised of two or more distinct cell populations with distinct borders. Desmoid fibromatosis (DF) is a rare musculoaponeurotic tissue tumor that grows deep in the connective tissue and shows locally aggressive behavior. Only two cases of collision tumors with desmoid fibromatosis are reported in the English literature, albeit papillary thyroid carcinoma with desmoid fibromatosis-like stroma is regarded as a variant rather than a collision tumor. We present a unique case of collision tumor with desmoid fibromatosis surrounding intra-abdominal metastasis from urothelial carcinoma. A 65-year-old white male with history of bladder and left renal pelvis high-grade papillary urothelial carcinoma status post-nephrectomy was found to have a small bowel obstruction due to a soft tissue mass. Histology of the mass showed multiple matted lymph nodes with metastatic urothelial carcinoma admixed with a proliferation of spindle cells positive for nuclear beta-catenin, consistent with desmoid fibromatosis. While the prior surgical site likely acted as a nidus for development of desmoid fibromatosis, we also hypothesize that a dysregulation of beta-catenin signaling pathways within the cancer cells might have attributed to the spindle cell proliferation in the stroma surrounding the tumor. Our case emphasized the importance of clinical suspicion of desmoid fibromatosis in patients with metastatic cancer, requiring a prompt diagnosis and treatment to decrease the risk of complications and local recurrence.
The role of project management in the success of green building projects: Egypt as a case study
Authors: Abdelkhalik Heba Farouk , Azmy Hisham Hussein 
Published: July 2022

AbstractSustainability and project management are two trends that have taken global interest in the last decades due to their significant role in various fields of life. However, these two topics have rarely been addressed in one study or framework. As sustainability and environmental issues are not specifically or systematically considered in most major project management frameworks such as the Project Management Body of Knowledge (PMBOK), Individual Competence Baseline (ICB), International Organization for Standardization (ISO 21500:2012), and so on. Furthermore, sustainability applications in the construction field under the term “green buildings” are facing various types of obstacles that obstruct the pervasion of this type of construction in an adequate and required way. Some of these obstacles have been addressed in recent studies with suggested solutions, but the role of project management in overcoming or even mitigating the risk of these obstacles was almost absent in most of these studies. Therefore, this paper attempts to observe the most important obstacles facing the application of sustainability in the construction field and taking the green construction situation in Egypt as a case study. In addition, this paper aims to investigate the role of project management in green building projects’ success, through project management best practices’ applications to overcome the main reasons that obstruct the green building projects movement. The results showed that there is a lack of management methods that address sustainable construction projects. In addition, there is no clear methodology governing the green building management process. Also, the unspecified responsibilities between stakeholders in green building projects lead to difficulties in managing and implementing green buildings. However, some defined obstructions could be overcome by project management’s best practices and methods.
Effectiveness of electrokinetic-enhanced oil recovery (EK-EOR): a systematic review
Authors: Ikpeka Princewill M. , Ugwu Johnson O. , Pillai Gobind G. , Russell Paul 
Published: July 2022

AbstractHydrocarbons continue to play an important role in providing affordable energy to meet rising energy demand. Amidst growing concerns on the environmental impact of oil and gas production processes, many researchers are increasingly exploring environmentally sustainable methods of extracting hydrocarbons from the reservoir. The introduction of direct current into the pore space activates mechanisms that enhance fluid flow, reduces produced water, decreases associated hydrogen sulfide production, and leaves no material footprint on the environment. Previous laboratory studies and field applications have reported varying degrees of success of the EK-EOR mechanism. However, the mechanism and effectiveness of this technique remain unclear. This systematic literature review provides an opportunity to critically evaluate laboratory results, establish a basis for the effectiveness of the EK-EOR mechanism and identify possible future research directions. In this study, 52 articles were identified and reviewed in a selection process that adhered to the PRISMA protocol. Data extracted from these articles were fed into the EK-EOR model, and Monte Carlo simulation (10,000 iterations) was used to determine the success rate of the EK-EOR process. Insights obtained from the simulation indicate that EK-EOR alone is not effective (with a success rate of 45%). Insights from published laboratory experiments indicate that interstitial clay affects the electro-osmotic permeability of reservoir rocks which determines the effectiveness of the EK-EOR mechanism. Salt deposition on the cathode and generation of gases (oxygen and chlorine at the anode) are significant limitations of the EK-EOR. The review concludes by identifying future areas of application of EK-EOR.
The 21-Gene Recurrence Score in Clinically High-Risk Lobular and Ductal Breast Cancer: A National Cancer Database Study
Authors: Abel Mary Kathryn , Shui Amy M. , Chien A. Jo , Rugo Hope S. , Melisko Michelle , Baehner Frederick , Mukhtar Rita A. 
Published: July 2022

AbstractObjectiveThe aim of this study was to evaluate whether patients with invasive lobular carcinoma (ILC) are more likely to have discordant clinical and genomic risk than those with invasive ductal carcinoma (IDC) when using the 21-gene recurrence score (RS), and to assess overall survival outcomes of patients with 1–3 positive nodes and RS ≤25 with and without chemotherapy, stratified by histology.MethodsWe performed a cohort study using the National Cancer Database and included patients with hormone receptor-positive, HER2-negative, stage I–III invasive breast cancer who underwent 21-gene RS testing. Our primary outcome was rate of discordant clinical and genomic risk status by histologic subtype. Propensity score matching was used to compare 60-month overall survival in individuals with 1–3 positive nodes and RS ≤25 who did and did not receive chemotherapy.ResultsOverall, 186,867 patients were included in our analysis, including 37,685 (20.2%) patients with ILC. There was a significantly higher rate of discordant clinical and genomic risk in patients with ILC compared with IDC. Among patients with 1–3 positive nodes and RS ≤25, there was no significant difference in survival between those who did and did not receive chemotherapy in the IDC or ILC cohorts. Unadjusted exploratory analyses of patients under age 50 years with 1–3 positive nodes and RS ≤25 showed improved overall survival in IDC patients who received chemotherapy, but not among those with ILC.ConclusionOur findings highlight the importance of lobular-specific tools for stratifying clinical and genomic risk, as well as the need for histologic subtype-specific analyses in randomized trials.
A Polish adaptation of the Self-Assessed Wisdom Scale (SAWS) in older adults
Authors: Brudek Paweł , Cyranka Katarzyna , Webster Jeffrey Dean 
Published: July 2022

Cel pracyW artykule przedstawiono wyniki prac nad polską adaptacją Samoopisowej Skali Mądrości (SAWS). Przedstawiono właściwości psychometryczne polskiej wersji narzędzia.MetodaBadanie miało charakter ilościowy i zostało przeprowadzone w schemacie korelacyjnym. Respondenci wypełnili zestaw kwestionariuszy. W badaniu wzięło udział 880 osób w wieku od 60 do 80 lat (M = 68,15, SD = 5,96). Oprócz SAWS zastosowano sześć innych metod psychologicznych. Dobór narzędzi pomiarowych był celowy.WynikiOstateczna polska wersja SAWS zawiera z 40 pozycji (w tym 36 diagnostycznych), które składają się na 5 wymiarów mądrości: (1) Doświadczenia życiowe, (2) Regulacja emocjonalna, (3) Reminiscencja i refleksyjność, (4) Otwartość i (5) Humor. Współczynnik rzetelności dla całej skali (36 pozycji) wyniósł a = 0,92 (bardzo wysoki). Rzetelności (α-Cronbach) dla poszczególnych skal waha się od α=0,60 do α=0,84. Trafność skali oceniano za pomocą analizy konfirmacyjnej.WnioskiWyniki są zgodne z pierwotną wersją skali, wykazano zatem, że polska wersja SAWS spełnia wymagania psychometryczne stawiane testom psychologicznym. Skalę z powodzeniem można stosować badaniach naukowych dotyczących osób starszych
PEDAGOGIA HISTÓRICO-CRÍTICA E A EMANCIPAÇÃO DO INDIVÍDUO
Authors: Chiappetta Tatiana 
Published: July 2022

Este estudo é uma revisão de literatura que objetiva compreender a pedagogia histórico-crítica como alternativa ao modelo de escola dominante. A partir desse pressuposto onde a escola, na sociedade burguesa, trabalha como um aparelho ideológico com o objetivo de disseminar o modo de produção capitalista. Ao mesmo tempo que forma mão-de-obra para o trabalho, internaliza no indivíduo o modo de vida burguesa. A pedagogia histórico-crítica (PHC) surge como uma proposta didática para romper essa ação reprodutivista e colaborar para formação de um indivíduo crítico e emancipado.
Gruppensolidarität zwischen Rationalität und Ritualität
Authors: Tranow Ulf 
Published: July 2022

Abstract
Group solidarity requires that members are committed to solidarity norms. Addressing the question of how such commitments are socially (re)produced, the starting point of this article is Lindenberg's Theory of Social Rationality (TSR) and its notion of solidarity frames. These frames consist of solidarity as a goal of action and associated stocks of knowledge and moral feelings. TSR makes it clear that solidarity frames are fragile and precarious. They must be regularly recharged with motivational energy and symbols in order to prevail over compet-ing motives. However, TSR cannot satisfactorily explain how this works. This shortcoming is addressed by Collins' Interaction Ritual Chains Theory (IRCT). It is argued that solidarity frames are a potential outcome of interaction rituals and it is clarified under which conditions this can be expected. A distinction is made between three essential types of rituals: identity rituals, governance rituals, and exchange rituals.
Sind Kollektive auf Anwesenheit angewiesen?
Authors: Landweer Hilge 
Published: July 2022

Abstract
This paper explores the role of co-presence in the emergence of collectives with reference to corporeal communication in online conferences and seminars. It emphasizes the importance of feelings of togetherness and demarcates them from feelings of mere affiliation. The emergence of shared feelings depends on corporeal communication, and specifically on the solidary form of corporeal communication as opposed to its antagonistic counterpart. Under online conditions, both forms tend to generate illusions, since a genuine exchange of glances, which opens up corporeal communication in the first place, is not possible. The missing exchange of glances must therefore be compensated online by imagination and the concentration on linguistic meanings.
Zwischen Identität und Diskurs – Wege zur Gemeinschaft?
Authors: Kaufmann Matthias 
Published: July 2022

Abstract
The article shows that with respect to increasing complaints about the social coldness of our current society and awareness of the urgent needs in the global south it should be clear that we need a moral motivation to solidarity beyond all legal regulations. While at first glance it may seem seducing to draw on the concept of community in the sense of Tönnies, it becomes quickly clear that this is much more dangerous but useful. As the application of universal ethics may be difficult in intercultural correlations, an interpretation of discourse ethics by Seyla Benhabib seems promising. The idea of identity may provide us with a number of insights, but it leads into a misinterpretation of democracy and, in the end, it is extremely dangerous for society if it is split into a growing number of identity groups.
The Treatment of Violence in Howard Barker’s Judith
Authors: Arf Pshtiwan M. , Abdulsalam Hamid B. 
Published: July 2022

As a postmodern dramatist, Howard Barker does not assume any presupposed meanings or messages of his writings. However, he deliberately and systematically works on deconstructing those ‘grand narratives’ that shape and give meaning to this world. In Judith, Barker challenges the audience’s understanding of history and their knowledge of the biblical story of Judith. His interest in the story is to defy traditional view towards the events, especially those which are related to religion, gender, history and violence. The current study aims at showing that seduction and extremity of violence which are at the heart of Barker’s Judith. It also shows how he connects violence to sex, seduction, and gender. Through these elements, Barker creates an atmosphere of uneasiness and bewilderment that necessitates deep thinking on the part of the audience. Barker uses: history to rewrite it, religion to reveal his faith in Christianity and violence to reveal it as human instinct and a necessity. In Barker’s play sex and violence are intermingled as two related sides that form greater part in staging catastrophe. The ultimate goal of this paper is to show Judith as the product of The Theater of Catastrophe which relates violence to gender and seduction. As a Biblical story the play embraces a great amount of violence which the researchers examine in detail; how he stages extremity and uses catastrophe to create meaning in his play.