bluuebunny/crossref_metadata_2025_split huggingface dataset:
West syndrome: a comprehensive review
Authors: Pavone Piero , Polizzi Agata , Marino Simona Domenica , Corsello Giovanni , Falsaperla Raffaele , Marino Silvia , Ruggieri Martino 
Published: August 2020

AbstractSince its first clinical description (on his son) by William James West (1793–1848) in 1841, and the definition of the classical triad of (1) infantile spasms; (2) hypsarrhythmia, and (3) developmental arrest or regression as “West syndrome”, new and relevant advances have been recorded in this uncommon disorder. New approaches include terminology of clinical spasms (e.g., infantile (IS) vs. epileptic spasms (ES)), variety of clinical and electroencephalographic (EEG) features (e.g., typical ictal phenomena without EEG abnormalities), burden of developmental delay, spectrum of associated genetic abnormalities, pathogenesis, treatment options, and related outcome and prognosis. Aside the classical manifestations, IS or ES may present with atypical electroclinical phenotypes (e.g., subtle spasms; modified hypsarrhythmia) and may have their onset outside infancy. An increasing number of genes, proteins, and signaling pathways play crucial roles in the pathogenesis. This condition is currently regarded as a spectrum of disorders: the so-called infantile spasm syndrome (ISs), in association with other causal factors, including structural, infectious, metabolic, syndromic, and immunologic events, all acting on a genetic predisposing background. Hormonal therapy and ketogenic diet are widely used also in combination with (classical and recent) pharmacological drugs. Biologically targeted and gene therapies are increasingly studied. The present narrative review searched in seven electronic databases (primary MeSH terms/keywords included West syndrome, infantile spasms and infantile spasms syndrome and were coupled to 25 secondary clinical, EEG, therapeutic, outcomes, and associated conditions terms) including MEDLINE, Embase, Cochrane Central, Web of Sciences, Pubmed, Scopus, and OMIM to highlight the past knowledge and more recent advances.
Prophylactic nimodipine treatment improves hearing outcome after vestibular schwannoma surgery in men: a subgroup analysis of a randomized multicenter phase III trial
Authors: Scheller Christian , Rampp Stefan , Leisz Sandra , Tatagiba Marcos , Gharabaghi Alireza , Ramina Kristofer F. , Ganslandt Oliver , Matthies Cordula , Westermaier Thomas , Antoniadis Gregor , Pedro Maria T. , Rohde Veit , von Eckardstein Kajetan , Scheller Konstanze , Strauss Christian 
Published: August 2020

AbstractA 2016 published randomized multicenter phase III trial of prophylactic nimodipine treatment in vestibular schwannoma surgery showed only a tendency for higher hearing preservation rates in the treatment group. Gender was not included in statistical analysis at that time. A retrospective analysis of the trial considering gender, preoperative hearing, and nimodipine treatment was performed. The treatment group received parenteral nimodipine from the day before surgery until the seventh postoperative day. The control group was not treated prophylactically. Cochlear nerve function was determined by pure-tone audiometry with speech discrimination preoperatively, during in-patient care, and 1 year after surgery and classified according to the Gardner-Robertson grading scale (GR). Logistic regression analysis showed a statistically significant effect for higher hearing preservation rates (pre- and postoperative GR 1–4) in 40 men comparing the treatment (n = 21) and the control (n = 19) groups (p = 0.028), but not in 54 women comparing 27 women in both groups (p = 0.077). The results were also statistically significant for preservation of postoperative hearing with pre- and postoperative GR 1–3 (p = 0.024). There were no differences in tumor sizes between the treatment and the control groups in men, whereas statistically significant larger tumors were observed in the female treatment group compared with the female control group. Prophylactic nimodipine is safe, and an effect for hearing preservation in 40 men with preoperative hearing ability of GR 1–4 was shown in this retrospective investigation. The imbalance in tumor size with larger tumors in females of the treatment group may falsely suggest a gender-related effect. Further investigations are recommended to clarify whether gender has impact on nimodipine’s efficacy.
N-glycosylation of the human β1,4-galactosyltransferase 4 is crucial for its activity and Golgi localization
Authors: Shauchuk Auhen , Szulc Bożena , Maszczak-Seneczko Dorota , Wiertelak Wojciech , Skurska Edyta , Olczak Mariusz 
Published: August 2020

Abstractβ1,4-galactosyltransferase 4 (B4GalT4) is one of seven B4GalTs that belong to CAZy glycosyltransferase family 7 and transfer galactose to growing sugar moieties of proteins, glycolipids, glycosaminoglycans as well as single sugar for lactose synthesis. Herein, we identify two asparagine-linked glycosylation sites in B4GalT4. We found that mutation of one site (Asn220) had greater impact on enzymatic activity while another (Asn335) on Golgi localization and presence of N-glycans at both sites is required for production of stable and enzymatically active protein and its secretion. Additionally, we confirm B4GalT4 involvement in synthesis of keratan sulfate (KS) by generating A375 B4GalT4 knock-out cell lines that show drastic decrease in the amount of KS proteoglycans and no significant structural changes in N- and O-glycans. We show that KS decrease in A375 cells deficient in B4GalT4 activity can be rescued by overproduction of either partially or fully glycosylated B4GalT4 but not with N-glycan-depleted B4GalT4 version.
Performance and Market Maturity in Mutual Funds: Is Real Estate Different?
Authors: MacGregor Bryan D. , Schulz Rainer , Zhao Yuan 
Published: August 2020

AbstractDespite the lack of convincing evidence that active investment fund managers add value, the number of actively-managed US mutual funds has increased substantially over the last 25 years. While non-sector diversified mutual funds have received much attention, sector funds, except real estate mutual funds (REMFs), have not. In this paper, we provide new and more robust evidence on the performance of active REMFs compared to all actively managed mutual funds. We use the Carhart four-factor model with an additional liquidity factor as a risk-adjusted benchmark. We use wild bootstrap methods to deal with small samples, non-normality and heteroscedasticity, and we control for the false discovery of significant results. For portfolios of fund types, we find evidence of both significant outperformance and underperformance, net of fees, during 1992-2016. We consider non-overlapping five-year and three-year periods and find very limited evidence of persistent outperformance. For individual funds, we find that, for both sector and diversified funds, net of fees, only 0.79% are skilled. We find persistence in skills for only two individual fund managers of diversified funds. We investigate the effects of the outsourcing of management and of team versus individual management. Outsourcing has no effect on performance of non-RE sector funds but, for cap-based funds and style-based funds, it has a negative effect. There is some evidence that this may also be true for REMFs. Team management has no effect for any types of funds. Overall, we conclude that REMFs are generally no different from other sector funds.
Moth assemblages within urban domestic gardens respond positively to habitat complexity, but only at a scale that extends beyond the garden boundary
Authors: Ellis Emilie E. , Wilkinson Tom L. 
Published: August 2020

Abstract‘Wildlife-friendly’ gardening is a dominant theme in the media that readily engages public attention. However, there is little empirical evidence of the ecological benefits of increased habitat quality of individual domestic gardens. This study uses light-trapping to examine the response of moth assemblages to domestic gardens that are assessed in terms of their habitat complexity (simple and complex) both within the garden and extending out to a 30 m radius that includes surrounding habitats. The results clearly show that moth assemblages were influenced by complex habitats (particularly increasing levels of the variable shrubs and decreasing levels of artificial surfaces), but only at a scale that extended beyond the garden boundary to include the surrounding area. In other words, neither the complexity of the habitat within the garden or the size of the garden had any influence on the abundance or diversity of the moth assemblage. These results have implications for both garden management and landscape planning – if domestic gardens are to be a useful component of strategies to reduce biodiversity loss within the urban environment then they should provide good habitat quality and be managed as a network of interconnected patches rather than as individual units.
How Does Mining Salinisation Gradient Affect the Structure and Functioning of Macroinvertebrate Communities?
Authors: Sowa Agnieszka , Krodkiewska Mariola , Halabowski Dariusz 
Published: August 2020

AbstractElevated salinity creates degrading conditions for the development of aquatic biota in different regions of the world. There is a need for research on freshwater salinisation in order to understand how this stressor alters ecosystem function and to predict changes in biodiversity globally. Such data are missing from Central Europe, and therefore, the presented study was performed in inland anthropogenic ponds with different salinity levels located in the second largest European hard coal basin. The researcher indicated a positive correlation between water salinity and the biomass and density of macrozoobenthos as well as the percentage of shredders and the abundance of alien species, whereas there was a decrease in taxa diversity and richness and the abundance of filtering and gathering collectors and predators along with increasing salinity. The survey showed that a high level of nutrients and organic matter were also significantly correlated with the distribution of the macroinvertebrate taxa and functional feeding groups. The conducted research confirmed that mining salinisation acts as a strong filter that shapes the biodiversity because it affects the composition, abundance, biomass and functional traits of benthic macroinvertebrates and significantly contributes to the invasion of alien species.
Effects of nasal high flow on sympathovagal balance, sleep, and sleep-related breathing in patients with precapillary pulmonary hypertension
Authors: Spiesshoefer Jens , Bannwitz Britta , Mohr Michael , Herkenrath Simon , Randerath Winfried , Sciarrone Paolo , Thiedemann Christian , Schneider Hartmut , Braun Andrew T. , Emdin Michele , Passino Claudio , Dreher Michael , Boentert Matthias , Giannoni Alberto 
Published: August 2020

Abstract
Background
In precapillary pulmonary hypertension (PH), nasal high flow therapy (NHF) may favorably alter sympathovagal balance (SVB) and sleep-related breathing through washout of anatomical dead space and alleviation of obstructive sleep apnea (OSA) due to generation of positive airway pressure.

Objectives
To investigate the effects of NHF on SVB, sleep, and OSA in patients with PH, and compare them with those of positive airway pressure therapy (PAP).

Methods
Twelve patients with PH (Nice class I or IV) and confirmed OSA underwent full polysomnography, and noninvasive monitoring of SVB parameters (spectral analysis of heart rate, diastolic blood pressure variability). Study nights were randomly split into four 2-h segments with no treatment, PAP, NHF 20 L/min, or NHF 50 L/min. In-depth SVB analysis was conducted on 10-min epochs during daytime and stable N2 sleep at nighttime.

Results
At daytime and compared with no treatment, NHF20 and NHF50 were associated with a flow-dependent increase in peripheral oxygen saturation but a shift in SVB towards increased sympathetic drive. At nighttime, NHF20 was associated with increased parasympathetic drive and improvements in sleep efficiency, but did not alter OSA severity. NHF50 was poorly tolerated. PAP therapy improved OSA but had heterogenous effects on SVB and neutral effects on sleep outcomes. Hemodynamic effects were neutral for all interventions.

Conclusions
In sleeping PH patients with OSA NHF20 but not NHF50 leads to decreased sympathetic drive likely due to washout of anatomical dead space. NHF was not effective in lowering the apnea-hypopnoea index and NHF50 was poorly tolerated.
Treatment Updates for Neuromuscular Channelopathies
Authors: Jitpimolmard Nantaporn , Matthews Emma , Fialho Doreen 
Published: August 2020

AbstractPurpose of reviewThis article aims to review the current and upcoming treatment options of primary muscle channelopathies including the non-dystrophic myotonias and periodic paralyses.Recent findingsThe efficacy of mexiletine in the treatment of myotonia is now supported by two randomised placebo-controlled trials, one of which utilised a novel aggregated n-of-1 design. This has resulted in licencing of the drug via orphan drug status. There is also good evidence that mexiletine is well tolerated and safe in this patient group without the need for intensive monitoring. A range of alternative antimyotonic treatment options include lamotrigine, carbamazepine and ranolazine exist with variable evidence base. In vitro studies have shown insight into reasons for treatment failure of some medications with certain genotypes opening the era of mutation-specific therapy such as use of flecainide. In the periodic paralyses, the ability of MRI to distinguish between reversible oedema and irreversible fatty replacement makes it an increasingly useful tool to guide and assess pharmacological treatment. Unfortunately, the striking efficacy of bumetanide in hypokalaemic periodic paralysis animal models was not replicated in a recent pilot study in humans.SummaryThe treatment of skeletal muscle channelopathies combines dietary and lifestyle advice together with pharmacological interventions. The rarity of these conditions remains a barrier for clinical studies but the example of the aggregated n-of-1 trial of mexiletine shows that innovative trial design can overcome these hurdles. Further research is required to test efficacy of drugs shown to have promising characteristics in preclinical experiments such as safinamide, riluzule and magnesium for myotonia or bumetanide for hypokalaemic periodic paralysis.
The role of restraint in fatal excited delirium: a research synthesis and pooled analysis
Authors: Strömmer Ellen M. F. , Leith Wendy , Zeegers Maurice P. , Freeman Michael D. 
Published: August 2020

AbstractThe purpose of the present study was to perform a comprehensive scientific literature review and pooled data risk factor analysis of excited delirium syndrome (ExDS) and agitated delirium (AgDS). All cases of ExDS or AgDS described individually in the literature published before April 23, 2020 were used to create a database of cases, including demographics, use of force, drug intoxication, mental illness, and survival outcome. Odds ratios were used to quantify the association between death and diagnosis (ExDS vs. AgDS) across the covariates. There were 61 articles describing 168 cases of ExDS or AgDS, of which 104 (62%) were fatal. ExDS was diagnosed in 120 (71%) cases, and AgDS in 48 (29%). Fatalities were more likely to be diagnosed as ExDS (OR: 9.9,p < 0.0001). Aggressive restraint (i.e. manhandling, handcuffs, and hobble ties)was more common in ExDS (ORs: 4.7, 14, 29.2, respectively,p < 0.0001) and fatal cases (ORs: 7.4, 10.7, 50, respectively, p < 0.0001). Sedation was more common in AgDS and survived cases (OR:11, 25, respectively,p < 0.0001). The results of the study indicate that a diagnosis of ExDS is far more likely to be associated with both aggressive restraint and death, in comparison with AgDS. There is no evidence to support ExDS as a cause of death in the absence of restraint. These findings are at odds with previously published theories indicating that ExDS-related death is due to an occult pathophysiologic process. When death has occurred in an aggressively restrained individual who fits the profile of either ExDS or AgDS, restraint-related asphyxia must be considered a likely cause of the death.
Do Criminals Live Faster Than Soldiers and Firefighters?
Authors: Kwiek Monika , Piotrowski Przemysław 
Published: August 2020

AbstractA high risk of morbidity-mortality caused by a harsh and unpredictable environment is considered to be associated with a fast life history (LH) strategy, commonly linked with criminal behavior. However, offenders are not the only group with a high exposure to extrinsic morbidity-mortality. In the present study, we investigated the LH strategies employed by two groups of Polish men: incarcerated offenders (N = 84) as well as soldiers and firefighters (N = 117), whose professions involve an elevated risk of injury and premature death. The subjects were asked to complete the Mini-K (used as a psychosocial LH indicator) and a questionnaire which included a number of biodemographic LH variables. Although biodemographic and psychosocial LH indicators should be closely linked with each other, the actual connection between them is unclear. Thus, this study was driven by two aims: comparing LH strategies in two groups of men with a high risk of premature morbidity-mortality and investigating the relationship between the biodemographic and psychosocial LH dimensions. The study showed that incarcerated men employed faster LH strategies than soldiers and firefighters, but only in relation to biodemographic variables (e.g., number of siblings, age of sexual initiation, life expectancy). No intergroup differences emerged regarding psychosocial LH indicators. Moreover, the correlation analysis showed a weak association between biodemographic and psychosocial LH indicators. The results strengthen the legitimacy of incorporating biodemographic LH traits into research models and indicate the need for further research on the accuracy of the Mini-K. The possible explanations for the intergroup differences in LH strategies are discussed.
Swimming pool heating technology: A state-of-the-art review
Authors: Li Yantong , Nord Natasa , Huang Gongsheng , Li Xin 
Published: August 2020

AbstractA large amount of heat is needed to maintain the thermal comfort of both indoor and outdoor swimming pools in cold seasons. This motivates the development of various heating technologies aiming to reduce energy use, as well as operating and investment costs. Although their development can be traced back to the 1960s, a comprehensive review of these technologies is lacking. Therefore, this paper presents a comprehensive review of the development of heating technologies for swimming pools. This review firstly introduces available heat transfer models that can be used to calculate or predict heat loss and heat gain for swimming pools. Then, different passive and active technologies are summarized. The active heating technologies used for indoor swimming pools include solar collector, heat pump, waste heat recovery, geothermal energy, and congregation technologies. The active heating technologies used for outdoor swimming pools include solar collector, heat pump, PCM storage, geothermal energy, biomass heater, and waste heat recovery technologies. A discussion is presented on the practical and possible heating techniques for swimming pool applications. Finally, through the reviewed literature, future research opportunities are identified, to guide researchers to investigate swimming pool heating systems with suitable and relevant technologies.
Food Supply Chains as Cyber-Physical Systems: a Path for More Sustainable Personalized Nutrition
Authors: Smetana Sergiy , Aganovic Kemal , Heinz Volker 
Published: August 2020

AbstractCurrent food system evolved in a great degree because of the development of processing and food engineering technologies: people learned to bake bread long before the advent of agriculture; salting and smoking supported nomad lifestyles; canning allowed for longer military marches; etc. Food processing technologies went through evolution and significant optimization and currently rely on minor fraction of energy comparing with initial prototypes. Emerging processing technologies (high-pressure, pulsed electric fields, ohmic heating, ultrasound) and novel food systems (cultured biomass, 3-D bioprinting, cyber-physical chains) try to challenge the existing chains by developing potentially more nutritious and sustainable food solutions. However, new food systems rely on low technology readiness levels and estimation of their potential future benefits or drawbacks is a complex task mostly due to the lack of integrated data. The research is aimed for the development of conceptual guidelines of food production system structuring as cyber-physical systems. The study indicates that cyber-physical nature of modern food is a key for the engineering of more nutritious and sustainable paths for novel food systems. Implementation of machine learning methods for the collection, integration, and analysis of data associated with biomass production and processing on different levels from molecular to global, leads to the precise analysis of food systems and estimation of upscaling benefits, as well as possible negative rebound effects associated with societal attitude. Moreover, such data-integrated assessment systems allow transparency of chains, integration of nutritional and environmental properties, and construction of personalized nutrition technologies.
Greg, ML – Machine Learning for Healthcare at a Scale
Authors: Lapadula Paola , Mecca Giansalvatore , Santoro Donatello , Solimando Luisa , Veltri Enzo 
Published: August 2020

AbstractThis paper introduces the Greg, ML platform, a machine-learning engine and toolset conceived to generate automatic diagnostic suggestions based on patient profiles. Greg, ML departs from many other experiences in machine learning for healthcare in the fact that it was designed to handle a large number of different diagnoses, in the order of the hundreds. We discuss the architecture that stands at the core of Greg, ML, designed to handle the complex challenges posed by this ambitious goal, and confirm its effectiveness with experimental results based on the working prototype we have developed. Finally, we discuss challenges and opportunities related to the use of this kind of tools in medicine, and some important lessons learned while developing the tool. In this respect, we underline that Greg, ML should be conceived primarily as a support for expert doctors in their diagnostic decisions, and can hardly replace humans in their judgment.