bluuebunny/crossref_metadata_2025_split huggingface dataset:
Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review
Authors: Pellikaan Karlijn , Nguyen Naomi Q C , Rosenberg Anna G W , Coupaye Muriel , Goldstone Anthony P , Høybye Charlotte , Markovic Tania , Grugni Graziano , Crinò Antonino , Caixàs Assumpta , Poitou Christine , Corripio Raquel , Nieuwenhuize Rosa M , van der Lely Aart J , de Graaff Laura C G 
Published: June 2023

Abstract

Context
Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expressed genes on chromosome 15q11.2-q13. As life expectancy of patients with PWS increases, age-related diseases like malignancies might pose a new threat to health.

Objective
To investigate the prevalence and risk factors of malignancies in patients with PWS and to provide clinical recommendations for cancer screening.

Methods
We included 706 patients with PWS (160 children, 546 adults). We retrospectively collected data from medical records on past or current malignancies, the type of malignancy, and risk factors for malignancy. Additionally, we searched the literature for information about the relationship between genes on chromosome 15q11.2-q13 and malignancies.

Results
Seven adults (age range, 18-55 years) had been diagnosed with a malignancy (acute lymphoblastic leukemia, intracranial hemangiopericytoma, melanoma, stomach adenocarcinoma, biliary cancer, parotid adenocarcinoma, and colon cancer). All patients with a malignancy had a paternal 15q11-13 deletion. The literature review showed that several genes on chromosome 15q11.2-q13 are related to malignancies.

Conclusion
Malignancies are rare in patients with PWS. Therefore, screening for malignancies is only indicated when clinically relevant symptoms are present, such as unexplained weight loss, loss of appetite, symptoms suggestive of paraneoplastic syndrome, or localizing symptoms. Given the increased cancer risk associated with obesity, which is common in PWS, participation in national screening programs should be encouraged.
The Pregnancy-Associated Plasma Protein-A (PAPP-A) Story
Authors: Conover Cheryl A , Oxvig Claus 
Published: June 2023

Abstract
Pregnancy-associated plasma protein-A (PAPP-A) was first identified in the early 1970s as a placental protein of unknown function, present at high concentrations in the circulation of pregnant women. In the mid-to-late 1990s, PAPP-A was discovered to be a metzincin metalloproteinase, expressed by many nonplacental cells, that regulates local insulin-like growth factor (IGF) activity through cleavage of high-affinity IGF binding proteins (IGFBPs), in particular IGFBP-4. With PAPP-A as a cell surface–associated enzyme, the reduced affinity of the cleavage fragments results in increased IGF available to bind and activate IGF receptors in the pericellular environment. This proteolytic regulation of IGF activity is important, since the IGFs promote proliferation, differentiation, migration, and survival in various normal and cancer cells. Thus, there has been a steady growth in investigation of PAPP-A structure and function outside of pregnancy. This review provides historical perspective on the discovery of PAPP-A and its structure and cellular function, highlights key studies of the first 50 years in PAPP-A research, and introduces new findings from recent years.
An Investigation of the Collaborations Between Educators and Vocational Rehabilitation Counselors in Providing Pre-Employment Transition Services
Authors: Zhang Dalun , Li Yi-Fan , Roberts Eric , Orsag Meagan , Maddalozzo Robert 
Published: June 2023

Abstract
Employment has historically provided a person with value and the ability to live a self-determined and independent life. Interagency collaborations have been found to play a key role in promoting employment for those with special needs. However, there is little research on identifying the needs of the field for collaborations between vocational rehabilitation and educational agencies. We conducted a series of eight focus groups in a large state in the United States and identified needs from educators and vocational rehabilitation counselors. Participants regularly noted that they needed increased collaboration and a need for additional training and education for all stakeholders. They also identified many barriers to this increased collaboration and training, including access to the community that they served, limitations on time for training, collaboration, and access, challenges in communication, including language limitations and trust, and limitations around the support that they received. On the other hand, they often felt supported by their administration, and, when collaboration was possible, their co-workers and external providers served as great resources.
Performance enhancement and emissions reduction in a diesel engine using oleander and croton biodiesel doped with graphene nanoparticles
Authors: Wambui Treza , Hawi Meshack , Njoka Francis , Kamau Joseph 
Published: June 2023

Biodiesel is considered a suitable substitute for petroleum diesel because it is renewable, environment-friendly, and has a low carbon footprint. However, its high density, high viscosity and low heating value prevents it from replacing petroleum diesel completely. This study investigates the performance and emission characteristics of a compression ignition engine operating on oleander and croton biodiesel doped with graphene nanoparticles. Five fuel samples are used, including diesel (D100), diesel - 80% blended with oleander and croton biodiesel - 20% (OCB20) and OCB20 dosed with graphene nanoparticles at mass fractions of 50 ppm (mg/L), 75 ppm (mg/L) and 100 ppm (mg/L), respectively. The chemical composition of biodiesel and graphene nanoparticles is analyzed using Fourier Transform Infrared (FTIR) spectroscopy while the morphology of the nanoparticles is analyzed using Scanning Electron Microscope (SEM). Engine tests reveal a significant improvement in brake thermal efficiency, especially at 75 ppm concentration which is 2.76% and 18.93% higher than diesel and OCB20, respectively, and a reduction in brake specific fuel consumption by 2.44% and 16.67% compared to diesel and OCB20, respectively. Carbon monoxide (CO) and unburnt hydrocarbon emissions (UHC) decreases for the 50 ppm sample, recording 8.58% and 21.65% reduction in CO and 52.2% and 50% in UHC compared to the diesel and OCB20, respectively. However, Oxides of Nitrogen (NOx) emissions increase. The results indicate that graphene nanoparticle-enhanced biodiesel can adequately substitute petroleum diesel, albeit with NOx reduction techniques.
Leadership Coaching and the Myth of the Rational Client
Authors: Forbes Raymond L. 
Published: June 2023

This paper will address the issue of whether or not the clients of leadership coaches think and act in completely rational ways. It explores the question using a lens derived from the fields of Behavioral Economics and the Brain Sciences. Beginning with a look at the origins of the idea of rationality, this work proceeds to consider what’s really at issue, why it matters, and the possible trap posed by assuming strong client rationality. The paper concludes by posing options for resolution of the rationality myth by exploring the concepts of heuristics, bias, anchoring and priming. The paper concludes with suggestions for what leadership coaches can actually do to improve their work with clients and a short summary of the main ideas.
The transport function of the human lymphatic system—A systematic review
Authors: Thorup Lene , Hjortdal Anders , Boedtkjer Donna B. , Thomsen Morten B. , Hjortdal Vibeke 
Published: June 2023

AbstractPhysiological properties and function of the lymphatic system is still somewhat of a mystery. We report the current knowledge about human lymphatic vessel contractility and capability of adaptation. A literature search in PubMed identified studies published January 2000–September 2022. Inclusion criteria were studies investigating parameters related to contraction frequency, fluid velocity, and lymphatic pressure in vivo and ex vivo in human lymphatic vessels. The search returned 2885 papers of which 28 met the inclusion criteria. In vivo vessels revealed baseline contraction frequencies between 0.2 ± 0.2 and 1.8 ± 0.1 min1, velocities between 0.008 ± 0.002 and 2.3 ± 0.3 cm/s, and pressures between 4.5 (range 0.5–9.2) and 60.3 ± 2.8 mm Hg. Gravitational forces, hyperthermia, and treatment with nifedipine caused increases in contraction frequency. Ex vivo lymphatic vessels displayed contraction frequencies between 1.2 ± 0.1 and 5.5 ± 1.2 min−1. Exposure to agents affecting cation and anion channels, adrenoceptors, HCN channels, and changes in diameter‐tension properties all resulted in changes in functional parameters as known from the blood vascular system. We find that the lymphatic system is dynamic and adaptable. Different investigative methods yields alternating results. Systematic approaches, consensus on investigative methods, and larger studies are needed to fully understand lymphatic transport and apply this in a clinical context.
Development and characterization of canine‐specific computational models to predict pulsatile arterial hemodynamics and ventricular‐arterial coupling
Authors: Hotek Julia C. , Chirinos Julio A. , Detwiler Theodore J. , Regan Hillary K. , Regan Christopher P. 
Published: June 2023

AbstractPulsatile hemodynamics analyses provide important information about the ventricular‐arterial system which cannot be inferred by standard blood pressure measurements. Pulse wave analysis (PWA), wave separation analysis (WSA), and wave power analysis (WPA) characterize arterial hemodynamics with limited preclinical applications. Integrating these tools into preclinical testing may enhance understanding of disease or therapeutic effects on cardiovascular function. We used a canine rapid ventricular pacing (RVP) heart failure model to: (1) Characterize hemodynamics in response to RVP and (2) assess analyses from flow waveforms synthesized from pressure compared to those derived from measured flow. Female canines (n = 7) were instrumented with thoracic aortic pressure transducers, ventricular pacing leads, and an ascending aortic flow probe. Data were collected at baseline, 1 week, and 1 month after RVP onset. RVP progressively reduced stroke volume (SV), the PWA SV estimator, and WSA and WPA pulsatility and wave reflection indices. Indices derived from synthesized flow exhibited similar directional changes and high concordance with measured flow calculations. Our data demonstrate the value of analytical hemodynamic methods to gain deeper insight into cardiovascular function in preclinical models. These approaches can provide complementary value to standard endpoints in evaluating potential effects of pharmaceutical agents intended for human use.
Risk assessment of rain-induced debris flow in the lower reaches of Yajiang River based on GIS and CF coupling models
Authors: Yanting Huang , Yonggang Guo 
Published: June 2023

Abstract
In order to reduce the adverse impact of debris flow disasters on engineering construction facilities and social security in the lower reaches of the Yajiang River, this article selected 11 risk assessment factors such as elevation, aspect, profile curvature, Relief, and rainfall to study the occurrence rule of debris flow in this area. The data of disaster factors caused by debris flow points were derived and analyzed in ArcGIS. Then, factor correlation test and factor sensitivity level were established. The coupling model of qualitative mathematical model (analytic Hierarchy Process, AHP), quantitative mathematical model (binary logistic regression [LR]), machine learning model (random forest RF), and certainty factor (CF) were, respectively, used to predict the risk of debris flow disaster in the study area. After comparison, it was found that the CF–LR model had the highest accuracy. The results show that the areas with high debris flow risk are mainly concentrated in the first half of the lower reaches of the Yajiang River and distributed along both sides of the river bank. The annual rainfall range of 600–700 mm is the critical water source saturation value of debris flow in the study area.
An experimental and numerical study to evaluate performance parameters of GIS circuit breaker during low current interruption
Authors: Rao Mandava Mohana , Abhinav Roy Sonali , Kiran Ravi 
Published: June 2023

Abstract
The basic philosophy of two-stage blast interrupter is combination of self-blast and puffer-interrupter principles. Gas circuit breaker (GCB) with two-stage blast interrupter has two gas chambers. High gas pressure in one chamber generates by using arc energy and in other chamber, by mechanical compression. In such type of breakers, interruption becomes quite difficult at low currents and hence optimization of gas chambers design is essential. In view of above, a numerical program has been developed to calculate SF6 gas pressure-rise in various interrupter volumes and gas flow rates across different passages of interrupter w.r.t. time. To validate model developed in the study, an experimental set-up is established and measured transient gas pressure-rise generated during 420 kV GCB operation. The effect of speed-time characteristics, stroke length and piston diameter on performance parameters of 420 kV GCB model is analyzed. Further, to understand gas flow pattern across insulated nozzle during GCB operation, authors developed a CFX model. By using this numerical model, effect of nozzle profile, gas flow passage areas and profile of gas discharge vents on transient gas pressure and Mach number distribution across inter-electrode gap and gas flow rates through various outlets has been discussed in detail in the paper.
Variation, gender and perception: the social meaning of Japanese linguistic variables
Authors: Sherwood Stacey , Shaw Jason A. , Kawahara Shigeto , Mailhammer Robert , Antoniou Mark 
Published: June 2023

AbstractThe social categories that characterize a speaker frequently correlate with the use of specific linguistic variables. Research suggests that such correlations are sometimes recognized as socially-indexed meanings. This study examines Japanese individuals’ attitudes toward variables that have been shown to correlate with the social category of gender in production. In particular, we contrast patterns of gendered variation that (i) have been prescriptively associated with speaker sex and (ii) tend to correlate with gender in speech production but are outside of the set of prescriptive “women’s language”. We found that individuals have formed associations between the gender of the speaker and prescriptive variables but not other patterns of variation. Additionally, knowledge of the speech context of the variables had no significant effect on individuals’ judgments. The results indicate that not all social information available from patterns of language use is recovered by listeners. More broadly, examining the transmission of social meaning through linguistic variation requires a combination of production- and perception-based research methods.
Immune checkpoint inhibitor-related pancreatitis: What is known and what is not
Authors: Jiang Chunyan , Tang Wen , Yang Xu , Li Hongwei 
Published: June 2023

Abstract
A 47-year-old man presented with a 1 week history of progressive fatigue and decreased appetite. He had stage IV oral squamous cell carcinoma and was treated with sintilimab, a programmed cell death protein 1 inhibitor, 22 months earlier. Laboratory work-up revealed significant elevation of bilirubin, liver enzymes, glucose, and lipase. Ultrasound examination and magnetic resonance cholangiopancreatography showed severe stenosis and occlusion of the pancreatic segment of the common bile duct, and PET/CT revealed swelling of the pancreas with diffuse increase in glucose metabolism. He was diagnosed with immune checkpoint inhibitor (ICI)-related pancreatitis and the treatment with sintilimab was permanently discontinued. He was administered systemic methylprednisolone at a dose of 2 mg/kg/day and subcutaneous insulin injection, without intravenous fluid or protease inhibitor. He improved quickly and received oral methylprednisolone for 10 months in gradually decreasing doses. He maintained well at 20 month follow-up. ICI-related pancreatitis is rare and varied. Further studies are needed to investigate the differences in the two types of ICI-related pancreatitis: acute pancreatitis and autoimmune pancreatitis-like cases.
Crystal structure of poly[bis(μ
2-2,6-bis(1-imidazoly)pyridine-κ
2
N:N′)copper(II)] diperchlorate dihydrate, C22H22Cl2CuN10O10
Authors: Kan Sujuan , Ma Yawei , Chen Chunjie , Qian Jun 
Published: June 2023

Abstract
C22H22Cl2CuN10O10, monoclinic, P21/c (no. 14), a = 6.8548(14) Å, b = 12.482(3) Å, c = 17.162(4) Å, β = 112.54(3)°, V = 1356.2(6) Å3, Z = 2, R

gt
(F) = 0.0575, wR

ref
(F
2) = 0.1726, T = 293 K.
Ecological knowledge, use and management of maax ik (Capsicum annuum var. glabriusculum (Dunal) Heiser & Pickersgill) in a rural Mayan community
Authors: Solís-Montero Virginia , Munguía-Rosas Miguel A. , Bello-Bedoy Rafael 
Published: June 2023

Background: Capsicum annuum var. glabriusculum is the ancestor of most of the economically important chiles in the world. It is widely distributed throughout Mexico, and currently all indigenous groups interact with this plant species. On the Yucatan Peninsula wild chile is known as maax ik by the Maya. Although this area has been recognized as a domestication and diversification center of chile, ethnobotanical research on this species is still limited.
Questions: What ecological knowledge do local people have about maax ik? Where and how is maax ik gathered and managed? What uses does maax ik currently have?
Studied species: Capsicum annuum var. glabriusculum
Study site and dates: San Bernardo, Yucatan. Summer-Autumn 2021.
Methods: In-depth semi-structured interviews and participatory observation. The management practices were identified, and management intensity was estimated.
Results: Informants knew that maax ik reproduces sexually and recognized the role of birds in its seed dispersal. Maax ikwas mainly gathered from homegardens where this species was tolerated and other non-selective incipient management practices such as protection and promotion were also carried out. Fruit was harvested without harming the plant and only a small portion of the available fruit was usually taken. The informants used maax ik essentially as food. The management intensity index was relatively high (5).
Conclusions: Maax ik is a sustenance food resource that is sustainably used and non-selectively managed in homegardens by our informants in San Bernardo, Yucatan.
A qualidade da água para o consumo humano em escolas da zona rural do município de Humaitá-AM
Authors: Farias Clenio Ferreira de , Galvão Maria Edenilda da Silva 
Published: June 2023

A escassez de recursos hídricos é um dos maiores problemas enfrentados pela comunidade global nos últimos tempos. A ausência de sensibilidade de algumas pessoas quanto ao uso racional da água pode comprometer a disponibilidade ou ainda sua qualidade. A qualidade da água é determinada por um conjunto de elementos que podem interferir nas propriedades da água tornando-a inconveniente para o consumo. Esta pesquisa objetivou analisar a qualidade da água em 3 escolas da zona rural do município de Humaitá-AM. Para aferição da qualidade foi utilizado o método padrão para o exame de água e águas residuais (SMWW, 23ª Edição, 2017). Os valores encontrados foram comparados com os dispositivos da Portaria de consolidação nº 5/GM/MS, de 28 de setembro de 2017, Resolução nº 357/CONAMA, de 17 de março de 2005, e Libânio (2010). Os resultados apresentaram baixa concentração de cloro residual livre nos sistemas das 3 escolas. Na escola 1, foi constatada também, a presença de coliformes totais nos 4 pontos de coleta (poço, reservatório, bebedouro e torneiras de cozinha) e a Presença de Escherichia coli no poço, reservatório e na torneira da cozinha. Na escola 2, os resultados apresentaram a presença de coliformes totais no poço e no reservatório. Outro parâmetro considerado fora do padrão foi o pH, o qual apresentou grau de acidez nas amostras das 3 escolas. Os resultados mostram contaminação microbiológica da água nas escolas 1 e 2, fato que põe em risco a saúde de alunos, servidores e outros usuários.
Palavras-Chave: Qualidade da água. Escolas da zona rural. Parâmetros de qualidade da água. Humaitá-AM.
Ecopedagogia na Contemporaneidade: Caminhos Percorridos e Perspectivas para Fomentar a Cultura da Sustentabilidade
Authors: Prass Andressa Amaro , Zucchetti Dinora Tereza , Wirth Júlia , Nobre Suelen Bomfim 
Published: June 2023

O movimento da Ecopedagogia propõe uma educação para a cidadania planetária que possibilite a construção e a consolidação de uma cultura da sustentabilidade, em uma perspectiva sistêmica, que considere a ecologia profunda e as biociências. É notório que a Ecopedagogia almeja oportunizar formações que contribuam para o aprimoramento das percepções ambientais de todos os cidadãos, integrando o ser humano e o ambiente, por meio de uma postura solidária e colaborativa. Neste cenário, este estudo visa compreender o conceito contemporâneo de Ecopedagogia, à luz de literatura especializada. Partindo desta premissa, apresenta-se uma pesquisa de natureza básica, de cunho qualitativo, com método científico dedutivo, bibliográfica e exploratória quanto aos procedimentos, uma vez que se baseia em estudos já publicados em bases eletrônicas, por pesquisadores da área de Educação Ambiental, no território nacional, no período de 2000-2023. Pode-se constatar que a concepção de Ecopedagogia ultrapassa propostas pedagógicas, compreende-a, também, como um novo paradigma educacional voltado para a valorização de todas as manifestações de vida do Planeta em perspectiva de uma pedagogia democrática, dialógica, solidária e problematizadora. Por fim, entende-se que a Ecopedagogia na atualidade apresenta conceitos atrelados a ideia de um movimento pedagógico e ato político, que articulam um projeto social global, para a promoção da cultura da sustentabilidade. Neste viés, as práticas educativas ecopedagógicas, tornam-se essenciais para minimizar a degradação ambiental e a exclusão social, contribuindo diretamente para a educação cidadã e formação do sujeito ecológico.
PALAVRAS-CHAVE: Ecopedagogia. Cidadania Planetária. Educação para a sustentabilidade. Cultura da Sustentabilidade.