bluuebunny/crossref_metadata_2025_split huggingface dataset:
Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients
Authors: Verhalleman Quinten , Richter Jute , Proesmans Marijke , Decaluwé Herbert , Debeer Anne , Van Raemdonck Dirk 
Published: September 2022

Abstract

OBJECTIVES
The purpose of this study was to evaluate the diagnosis of, clinical signs of and strategy for congenital cystic adenomatoid malformations (CCAM).

METHODS
In this retrospective study, patients who had thoracic surgery for CCAM lesions at the University Hospitals of Leuven from July 1993 to July 2016 were identified. Data on diagnosis, prenatal ultrasound findings, clinical signs, lesion site, CCAM type, associated anomalies, imaging, surgical approach and postoperative morbidity were reviewed. The Fisher exact and Mann–Whitney tests were used as appropriate.

RESULTS
A total of 55 patients were identified with CCAM. In 65% (n = 36/55), CCAM was detected on prenatal ultrasound scans. Prenatal symptoms due to hydrops or mass effect were present in 22% (n = 8/36), 6 of whom eventually needed prenatal intervention (thoracoamniotic shunting or intrauterine puncture). Elective surgery was performed in 40% of patients (n = 22/55); others developed clinical signs that indicated the need for semi-urgent surgery, with clinical signs of pulmonary infection and respiratory distress being the most common. Most patients had a single lobectomy via a minithoracotomy. Postoperative complications and length of stay were significantly higher in patients with CCAM with preoperative clinical signs.

CONCLUSIONS
Surgery in asymptomatic patients with CCAM can be performed safely with few postoperative complications and can be planned at a young age in patients with a high risk of developing clinical signs later in life.
SpanishHTTgene study reveals haplotype and allelic diversity with possible implications for germline expansion dynamics in Huntington disease
Authors: Ruiz de Sabando Ainara , Urrutia Lafuente Edurne , Galbete Arkaitz , Ciosi Marc , García Amigot Fermín , García Solaesa Virginia ,   Spanish HD Collaborative group, Martínez Victoria Álvarez , Martinez-Descals Asunción , Mila Montserrat , Trujillo-Tiebas Maria José , López-Sendón Jose Luis , Fenollar-Cortés María , Legarda Inés , Noguera Sara Bernal , Millán Jose M , Durán-Herrera Camen , Monckton Darren G , Ramos-Arroyo Maria A 
Published: September 2022

AbstractWe aimed to determine the genetic diversity and molecular characteristics of the Huntington disease (HD) gene (HTT) in Spain. We performed an extended haplotype and exon one deep sequencing analysis of the HTT gene in a nationwide cohort of population-based controls (n = 520) and families with symptomatic individuals referred for HD genetic testing. This group included 331 HD cases and 140 carriers of intermediate alleles. Clinical and family history data were obtained when available. Spanish normal alleles are enriched in C haplotypes (40.1%), whereas A1 (39.8%) and A2 (31.6%) prevail among intermediate and expanded alleles, respectively. Alleles ≥ 50 CAG repeats are primarily associated with haplotypes A2 (38.9%) and C (32%), which are also present in 50% and 21.4%, respectively, of HD families with large intergenerational expansions. Non-canonical variants of exon one sequence are less frequent, but much more diverse, in alleles of ≥27 CAG repeats. The deletion of CAACAG, one of the six rare variants not observed among smaller normal alleles, is associated with haplotype C and appears to correlate with larger intergenerational expansions and early onset of symptoms. Spanish HD haplotypes are characterized by a high genetic diversity, potentially admixed with other non-Caucasian populations, with a higher representation of A2 and C haplotypes than most European populations. Differences in haplotype distributions across the CAG length range support differential germline expansion dynamics, with A2 and C showing the largest intergenerational expansions. This haplotype-dependent germline instability may be driven by specific cis-elements, such as the CAACAG deletion.
The GBA variant E326K is associated with alpha-synuclein aggregation and lipid droplet accumulation in human cell lines
Authors: Smith Laura J , Bolsinger Magdalena M , Chau Kai-Yin , Gegg Matthew E , Schapira Anthony H V 
Published: September 2022

Abstract
Sequence variants or mutations in the GBA gene are numerically the most important risk factor for Parkinson disease (PD). The GBA gene encodes for the lysosomal hydrolase enzyme, glucocerebrosidase (GCase). GBA mutations often reduce GCase activity and lead to the impairment of the autophagy-lysosomal pathway, which is important in the turnover of alpha-synuclein, accumulation of which is a key pathological hallmark of PD. Although the E326K variant is one of the most common GBA variants associated with PD, there is limited understanding of its biochemical effects. We have characterized homozygous and heterozygous E326K variants in human fibroblasts. We found that E326K variants did not cause a significant loss of GCase protein or activity, endoplasmic reticulum (ER) retention or ER stress, in contrast to the L444P GBA mutation. This was confirmed in human dopaminergic SH-SY5Y neuroblastoma cell lines overexpressing GCase with either E326K or L444P protein. Despite no loss of the GCase activity, a significant increase in insoluble alpha-synuclein aggregates in E326K and L444P mutants was observed. Notably, SH-SY5Y overexpressing E326K demonstrated a significant increase in the lipid droplet number under basal conditions, which was exacerbated following treatment with the fatty acid oleic acid. Similarly, a significant increase in lipid droplet formation following lipid loading was observed in heterozygous and homozygous E326K fibroblasts. In conclusion, the work presented here demonstrates that the E326K mutation behaves differently to the common loss of function GBA mutations; however, lipid dyshomeostasis and alpha-synuclein pathology are still evident.
Blood transcriptomic biomarkers of alcohol consumption and cardiovascular disease risk factors: the Framingham Heart Study
Authors: Ma Jiantao , Huang Allen , Yan Kaiyu , Li Yi , Sun Xianbang , Joehanes Roby , Huan Tianxiao , Levy Daniel , Liu Chunyu 
Published: September 2022

Abstract

Background
The relations of alcohol consumption and gene expression remain to be elucidated.

Materials and Methods
We examined cross-sectional associations between alcohol consumption and whole blood derived gene expression levels and between alcohol-associated genes and obesity, hypertension, and diabetes in 5531 Framingham Heart Study (FHS) participants.

Results
We identified 25 alcohol-associated genes. We further showed cross-sectional associations of 16 alcohol-associated genes with obesity, nine genes with hypertension, and eight genes with diabetes at P &lt; 0.002. For example, we observed decreased expression of PROK2 (β = −0.0018; 95%CI: −0.0021, −0.0007; P = 6.5e − 5) and PAX5 (β = −0.0014; 95%CI: −0.0021, −0.0007; P = 6.5e − 5) per 1 g/day increase in alcohol consumption. Consistent with our previous observation on the inverse association of alcohol consumption with obesity and positive association of alcohol consumption with hypertension, we found that PROK2 was positively associated with obesity (OR = 1.42; 95%CI: 1.17, 1.72; P = 4.5e − 4) and PAX5 was negatively associated with hypertension (OR = 0.73; 95%CI: 0.59, 0.89; P = 1.6e − 3). We also observed that alcohol consumption was positively associated with expression of ABCA13 (β = 0.0012; 95%CI: 0.0007, 0.0017; P = 1.3e − 6) and ABCA13 was positively associated with diabetes (OR = 2.57; 95%CI: 1.73, 3.84; P = 3.5e − 06); this finding, however, was inconsistent with our observation of an inverse association between alcohol consumption and diabetes.

Conclusions
We showed strong cross-sectional associations between alcohol consumption and expression levels of 25 genes in FHS participants. Nonetheless, complex relationships exist between alcohol-associated genes and CVD risk factors.
Out of Obscurity: Local Leadership and Cultural Wealth in the Radical Communities of the West Riding Textile District, 1825–40
Authors: Sanders John 
Published: September 2022

Abstract
This paper revisits the local leadership of the various inter-related campaigns, agitations and oppositional activities that animated many of the communities of the West Riding textile district during the 1825–40 period. After examining different levels and types of leadership, it explores how people became leaders, the attractions and drawbacks of their role, and the challenges they faced. The study argues that the emergence of a generation of leaders who tapped into and contributed to the cultural wealth of their communities helps to explain the vitality of popular radicalism and the easy replenishment of early Chartist leadership in these localities.
Decreased GABA+ Levels in the Medial Prefrontal Cortex of Perimenopausal Women: A 3T 1H-MRS Study
Authors: Tran Kim H , Luki Jessica , Hanstock Sarah , Hanstock Christopher C , Seres Peter , Aitchison Katherine , Shandro Tami , Le Melledo Jean-Michel 
Published: September 2022

Abstract

Objective
Perimenopause is associated with an increased risk of developing a major depressive (MD) episode. A significant number of women develop their first MD episode during perimenopause, suggesting a unique pathophysiology of perimenopausal (PM) depression. Previous research has shown that depression is associated with decreased gamma-aminobutyric acid (GABA) levels in the medial prefrontal cortex (MPFC) of MD patients. The objective of this study was to compare MPFC GABA+ levels in healthy reproductive-aged (RD) and PM women.

Methods
A total of 18 healthy PM and 20 RD women were included in the study. MPFC GABA+ levels, which include homocarnosine and macromolecules, were measured via magnetic resonance spectroscopy using a 3 Tesla magnet. MPFC GABA+ levels were referenced to creatine + phosphocreatine (Cr+PCr). Absence of current or past psychiatric diagnosis was confirmed via a structured interview. RD participants were scanned during the early follicular phase of the menstrual cycle. PM women were scanned outside of ovulatory cycles.

Results
Mean MPFC GABA+ concentrations (relative to Cr+PCr) were decreased in the PM group compared with the RD group (PM mean = 0.08 ± 0.02, RD mean = 0.09 ± 0.02, t = −2.03, df = 36, P = .05) even after correcting for in percentage in gray matter (GM). Because PM women were inherently older than RD women (aged 48.8 ± 3.55 and 31.5 ± 9.66 years, respectively), the age difference between the 2 groups was statistically significant (P &lt; .001). When age was treated as an independent covariate and included in the model, the difference in GABA+ between PM and RD women was no longer significant (P = .092).

Conclusion
Perimenopause is associated with decreased MPFC GABA+/Cr+PCr levels, which may contribute to the increased risk of experiencing a MD episode during PM.
What is the Preferred Educational Background of Forensic Scientists?
Authors: Marohl Mark , Jensen Grace , Barkholtz Heather 
Published: September 2022

Abstract
Forensic science is a broad and rapidly developing field where fundamental scientific principles and techniques are used to investigate crimes. As the field evolves, universities offering forensic science degrees are increasing in both number and diversity of curricula. Paramount to any discussion around forensic science education is an assessment of what educational degrees are preferred by those hiring both entry- and supervisory-level forensic scientists. Quantification of degree preference through surveys of hiring officials occurred in 1986 and 1996. Since then, the Forensic Science Education Programs Accreditation Commission was established and has flourished, potentially enhancing the desirability of forensic science degrees. An updated survey was conducted to identify current educational background preferences within the field. The survey was distributed to members of the American Society of Crime Laboratory Directors and members of the Society of Forensic Toxicologists in hiring or leadership roles, yielding 221 responses. Results indicated a continued preference for a degree combination of master’s in forensic science and a bachelor’s degree in physical or biological sciences for both entry- and supervisory-level candidates. Holding only a bachelor’s in the physical or biological sciences was also preferred for entry-level candidates whereas the preference for supervisory-level candidates was strongly influenced by the respondents’ terminal degree.
The Significance of Data-Sharing Policy
Authors: Askarov Zohid , Doucouliagos Anthony , Doucouliagos Hristos , Stanley T D 
Published: September 2022

AbstractWe assess the impact of mandating data-sharing in economics journals on two dimensions of research credibility: statistical significance and excess statistical significance (ESS). ESS is a necessary condition for publication selection bias. Quasi-experimental difference-in-differences analysis of 20,121 estimates published in 24 general interest and leading field journals shows that data-sharing policies have reduced reported statistical significance and the associated t-values. The magnitude of this reduction is large and of practical significance. We also find suggestive evidence that mandatory data-sharing reduces ESS and hence decreases publication bias.
The Marquess and Marchioness of Buckingham, Sir Joshua Reynolds, and the eighteenth-century context for Rembrandt’s Bellona in the Metropolitan Museum of Art
Authors: Morgan Andrea 
Published: September 2022

Abstract
Rembrandt’s style has often been seen as antithetical to the aesthetics of classicism. Yet, in the eighteenth century – a period that saw a revival of those very principles – the English enthusiastically collected works attributed to the Dutch master. This paper considers George and Mary Nugent-Temple-Grenville, 1st Marquess and Marchioness of Buckingham, and their acquisition of Rembrandt’s imagined portrait of the goddess Bellona. Their artistic taste, like that of many of their contemporaries, has been described as strictly classical, hence the reasons for their interest in Rembrandt are examined here. In fact, Bellona was recommended to the Marquess and Marchioness by Sir Joshua Reynolds, himself well known for his affinity with the artist. The relationship between Reynolds and the Temple-Grenville family of this painting is explored and the acquisition situated within an eighteenth-century context and the vogue for portraying contemporary women in the guise of mythological figures, just as Rembrandt did in his depiction of Bellona.
Assessment of Physician Knowledge, Attitudes, and Practice for Lyme Disease in a Low-Incidence State
Authors: Brummitt Sharon I , Harvey Danielle J , Smith Woutrina A , Barker Christopher M , Kjemtrup Anne M 
Published: September 2022

Abstract
Lyme disease (LD), caused by the bacterium Borrelia burgdorferi, is transmitted to humans in California through the bite of infected blacklegged ticks (Ixodes pacificus). Overall, the incidence of LD in California is low: approximately 0.2 confirmed cases per 100,000 population. However, California’s unique ecological diversity results in wide variation in local risk, including regions with local foci at elevated risk of human disease. The diagnosis of LD can be challenging in California because the prior probability of infection for individual patients is generally low. Combined with nonspecific symptoms and complicated laboratory testing, California physicians need a high level of awareness of LD in California to recognize and diagnose LD efficiently. This research addresses an under-studied area of physicians’ knowledge and practice of the testing and treatment of LD in a low-incidence state. We assessed knowledge and practices related to LD diagnosis using an electronic survey distributed to physicians practicing in California through mixed sampling methods. Overall, responding physicians in California had a general awareness of Lyme disease and were knowledgeable regarding diagnosis and treatment. However, we found that physicians in California could benefit from further education to improve test-ordering practices, test interpretation, and awareness of California’s disease ecology with elevated levels of focal endemicity, to improve recognition, diagnosis, and treatment of LD in California patients.
Advanced observation of brain and nerve cells using two-photon microscopy with novel techniques
Authors: Ishii Hirokazu , Takahashi Taiga , Yamaguchi Kazushi , Nemoto Tomomi 
Published: September 2022

AbstractTwo-photon excitation fluorescence microscopy [two-photon microscopy (2PM)] is a robust technique for understanding physiological phenomena from the cellular to tissue level, attributable to the nonlinear excitation process induced by near-infrared ultrashort laser light pulses. Recently, we have been promoting the use of semiconductor lasers, adaptive optics, vector beams and nanomaterials to improve the observation depth or spatial resolution. The developed semiconductor-based laser light source successfully visualized the structure of the enhanced yellow fluorescent protein (EYFP)-expressing neurons at the hippocampal dentate gyrus without resecting the neocortex and neuronal activity in the hippocampal cornu ammonis (CA1) region in anesthetized mice at video rates. We also proposed using fluoropolymer nanosheets of 100-nm thickness for in vivo imaging and realized a wide field of view during anesthetized mouse brain imaging of 1-mm depth. Furthermore, the developed adaptive optical 2PM visualized single dendritic spines of EYFP-expressing neurons in cortical layer V of the secondary motor cortex, which had been difficult to observe due to the curvature of the brain surface. In addition, we combined 2PM and stimulated emission depletion microscopy to improve spatial resolution. This combined microscopy is noninvasive and has a superior spatial resolution, exceeding the diffraction limit of the conventional light. In this review, we describe our recent results and discuss the future of 2PM.
Improving biobehavioral health in younger breast cancer survivors: Pathways to Wellness trial secondary outcomes
Authors: Bower Julienne E , Partridge Ann H , Wolff Antonio C , Cole Steve W , Irwin Michael R , Thorner Elissa D , Joffe Hadine , Petersen Laura , Crespi Catherine M , Ganz Patricia A 
Published: September 2022

Abstract

Background
The Pathways to Wellness trial tested the efficacy of 2 interventions for younger breast cancer survivors: mindful awareness practices (MAPs) and survivorship education (SE). This planned secondary analysis examines intervention effects on stress, positive psychological outcomes, and inflammation (Clincaltrials.gov NCT03025139).

Methods
Women diagnosed with breast cancer at or before age 50 years who had completed treatment and had elevated depressive symptoms were randomly assigned to 6 weeks of MAPs, SE, or wait-list control (WLC). Assessments conducted at pre- and postintervention and at 3- and 6-month follow-up measured general stress perceptions, cancer-related intrusive thoughts and worry, positive affect, meaning and peace in life, altruism and empathy, and markers of inflammation. Analyses compared change in outcomes over time in each intervention group relative to WLC using linear mixed models.

Results
A total 247 women were randomly assigned to MAPs (n = 85), SE (n = 81), or WLC (n = 81). MAPs statistically significantly decreased intrusive thoughts and worry at postintervention and 3-month follow-up relative to WLC (P &lt; .027) and statistically significantly increased positive affect and meaning and peace at postintervention, with positive affect persisting at 3-month follow-up (P &lt; .027). SE statistically significantly decreased intrusive thoughts at 3-month follow-up and statistically significantly increased positive affect at 6-month follow-up relative to WLC (P &lt; .01). Proinflammatory gene expression increased in WLC relative to MAPs (P = .016) but did not differ from SE. There were no intervention effects on other outcomes.

Conclusion
MAPs had beneficial effects on psychological and immune outcomes in younger breast cancer survivors and is a promising approach for enhancing biobehavioral health.
Exploring the relationship between nursing work environment and medical error reporting among Jordanian nurses: a cross-sectional study
Authors: Dalky Alaa , Alolayyan Main , Abuzaid Sajeda , Abuhammad Sawsan 
Published: September 2022

Abstract

Objectives
This study was conducted to explore the relationship between the nursing work environment and medical error reporting practices among Jordanian nurses.

Methods
This study was a cross-sectional survey of 334 participants who were conveniently selected from three types of hospitals (private, teaching and public) in Amman city, Jordan. The data were collected using a standardized questionnaire which was adopted from previous studies and consisted of three main sections including demographics, nursing work environment (The Practice Environment Scale-Nursing Work Index) and medical error reporting practices (incident reporting practice scale). SPSS version 26 was used for data analysis.

Key findings
The study findings detected a strong positive relationship between the nursing work environment and medical error reporting practices. ‘Nurse’s participation in hospital affairs’ showed the highest impact on medical error reporting practices among nurses. Based on the regression model, the nursing work environment explained 65.1% of variations in nurses’ medical error reporting practices. It was found that medical error reporting practices were statistically different across marital status and hospital type.

Conclusions
Based on data analysis findings, the nursing work environment was statistically strongly correlated to medical error reporting practices. To improve medical error reporting practices among nurses, decision-makers and hospital administrators should redesign their nursing work environment to create a more positive and favourable work environment.
Jejunojejunal intussusception of a sutured enterotomy site after takedown and primary repair of persistent enterocutaneous fistula: a case report
Authors: Marlor Derek , Noor Sibat , Beck Justin , Taghlabi Khaled M , Al-Kasspooles Mazin 
Published: September 2022

Abstract
Enterocutaneous fistula (ECF) is a common complication of many abdominal surgeries. Although most ECF resolve spontaneously, there are many factors that can lead to persistence of the fistula. Management of persistent enterocutaneous fistula usually involves surgery with recurrence of fistula being the most common complication. Here we describe a case of 67-year-old female who presented with intussusception following repair of a persistent enterocutaneous. Given the rare finding of intussusception in adults, this case report presents an interesting complication.