bluuebunny/crossref_metadata_2025_split huggingface dataset:
Advances in the gene therapy of monogenic blood cell diseases
Authors: Bueren Juan A. , Quintana‐Bustamante Oscar , Almarza Elena , Navarro Susana , Río Paula , Segovia José C. , Guenechea Guillermo 
Published: June 2019

AbstractHematopoietic gene therapy has markedly progressed during the last 15 years both in terms of safety and efficacy. While a number of serious adverse events (SAE) were initially generated as a consequence of genotoxic insertions of gamma‐retroviral vectors in the cell genome, no SAEs and excellent outcomes have been reported in patients infused with autologous hematopoietic stem cells (HSCs) transduced with self‐inactivated lentiviral and gammaretroviral vectors. Advances in the field of HSC gene therapy have extended the number of monogenic diseases that can be treated with these approaches. Nowadays, evidence of clinical efficacy has been shown not only in primary immunodeficiencies, but also in other hematopoietic diseases, including beta‐thalassemia and sickle cell anemia. In addition to the rapid progression of non‐targeted gene therapies in the clinic, new approaches based on gene editing have been developed thanks to the discovery of designed nucleases and improved non‐integrative vectors, which have markedly increased the efficacy and specificity of gene targeting to levels compatible with its clinical application. Based on advances achieved in the field of gene therapy, it can be envisaged that these therapies will soon be part of the therapeutic approaches used to treat life‐threatening diseases of the hematopoietic system.
The effect of supportive care in preventing peri‐implant diseases and implant loss: A systematic review and meta‐analysis
Authors: Lin Cho‐Ying , Chen Zhaozhao , Pan Whei‐Lin , Wang Hom‐Lay 
Published: June 2019

AbstractObjectiveTo evaluate the influence of supportive treatment (SPT) during a maintenance period after implant placement on implant survival rate (SR) and incidence of peri‐implant diseases.Material and methodsA systemic literature search for studies published up to June 2018 was conducted by two independent reviewers using Pubmed/MEDLINE, EMBASE, and Cochrane Central databases. Clinical controlled trials (CCT) involved in SPT protocol with more than 1‐year follow‐up were included. Quantitative meta‐analyses were carried out to analyze the risk ratio (RR) of SR, the incidence of peri‐implantitis, and peri‐implant mucositis between SPT and non‐SPT groups. Any potential confounding factors were investigated using meta‐regression.ResultsNine CCTs fulfilled the criteria. To evaluate the influence of SPT on SR, peri‐implantitis, and peri‐implant mucositis, six of nine, three of nine, and three of nine articles were included in further meta‐analysis, respectively. SPT group significantly showed higher SR (RR: 1.10; p < 0.001), lower prevalence of peri‐implantitis (RR: 0.25; p < 0.001) and peri‐implant mucositis (RR: 0.57; p < 0.001) than the non‐SPT group. Meta‐regression of the selected studies failed to find an association between SR, peri‐implantitis, and peri‐implant mucositis and confounding factors: application of chemical agents and the frequency of SPT.ConclusionSPT can potentially improve peri‐implant health in terms of SR, peri‐implantitis, and peri‐implant mucositis. Additionally, the correlation in recall interval and adjunctive use of chemical agents during SPT to peri‐implant diseases and implant loss could not be found.
Cytopathological spectrum of peripheral neuroblastic tumours in fine needle aspiration cytology and categorisation as per International Neuroblastoma Pathology Classification
Authors: Koshy Abin , Jain Richa , Srinivasan Radhika , Bhatia Prateek , Kakkar Nandita , Rajwanshi Arvind , Gupta Nalini , Dey Pranab , Trehan Amita , Bansal Deepak 
Published: June 2019

AbstractObjectiveThe aim of this analysis was to describe the cytopathology spectrum of peripheral neuroblastic tumours (NTs) including neuroblastoma (NB), ganglioneuroblastoma (GNB) and ganglioneuroma (GN). Feasibility of applying the International Neuroblastoma Pathology Classification (INPC) to further subtype NTs in cytology was evaluated.MethodsAll peripheral NTs reported on fine needle aspiration during 2011‐2015 were retrieved and detailed cytomorphological evaluation was performed. Based on INPC criteria, NBs were further categorised as undifferentiated, poorly differentiated and differentiating subtypes. Mitotic‐karyorrhectic index was evaluated. Immunocytochemistry on cell blocks was reviewed wherever available. MYCN amplification by fluorescence in situ hybridisation was performed in 11 cases on smears.ResultsA total of 90 cases including 83 NBs, six GNB and one GN were evaluated. The age range was 12 days‐12 years, with 55 males and 45 females. Both the primary and metastatic locations were aspirated. Applying the INPC criteria, there were 61 poorly differentiated, 14 undifferentiated, eight differentiating NB and six GNB. Immunocytochemistry on cell blocks showed positivity for at least two neuronal markers in NB. Mitotic‐karyorrhectic index was high in 63, low in 22 and intermediate in two cases, respectively. MYCN amplification by fluorescence in situ hybridisation was feasible on smears and was amplified in 6 out of 11 cases tested.ConclusionPeripheral NT types including NB, GNB and GN have distinctive cytomorphology. NBs can be further subtyped as undifferentiated, poorly differentiated and differentiating subtypes as per INPC criteria.
The Arctic/Swedish APP mutation alters the impact of chronic stress on cognition in mice
Authors: Cortese Aurelio , Delgado‐Morales Raul , Almeida Osborne F. X. , Romberg Carola 
Published: June 2019

AbstractChronic stress is a major risk factor for developing Alzheimer's disease (AD) and promotes the processing of amyloid precursor protein (APP) to β‐amyloid (Aβ). However, the precise relationship of stress and disease‐typical cognitive decline is presently not well understood. The aim of this study was to investigate how early life stress may affect cognition in adult mice with and without soluble Aβ pathology typical for the early stages of the disease. We focussed on sustained attention and response control, aspects of cognition mediated by the prefrontal cortex that are consistently impaired both in early AD and after chronic stress exposure. Young wild‐type mice as well as transgenic arcAβ mice overexpressing the hAPParc/swe transgene were exposed to a chronic unpredictable stress paradigm (age 3–8 weeks). At 15 weeks, these mice were tested on the 5‐choice serial reaction time task, a test of sustained attention and executive control. We found that, expectedly, chronic stress increased impulsive choices and impaired sustained attention in wild‐type mice. However, the same treatment reduced impulsivity and did not interfere with sustained attention in arcAβ mice. These findings suggest an unexpected interaction between chronic stress and Aβ whereby Aβ‐pathology caused by the hAPParc/swe mutation prevented and/or reversed stress‐induced cognitive changes through mechanisms that deserve further investigation. They also indicate that Aβ, in modest amounts, may have a beneficial role for cognitive stability, for example by protecting neural networks from the impact of further physiological or behavioural stress.
Surgical treatment of a mandibular ameloblastic carcinoma with metastases to the mandibular lymph nodes in a pony
Authors: Derham A. M. , Kushnir A. , Hoey S. , Jahns H. , Hollis A. R. , Kearney C. M. 
Published: June 2019

SummaryA 4‐year‐old Connemara filly was presented with a rapidly growing oral mass on the right rostrolateral mandible and a right mandibular lymphadenopathy. Radiographs of the rostral mandible revealed a lytic, infiltrative mass consistent with soft tissue and mineralised material, and displacement of tooth 403. A biopsy showed characteristic histopathological features of an ameloblastic carcinoma. Subsequent FNA of the right abnormally firm and enlarged mandibular lymph node confirmed the metastatic spread. Computed tomography of the head was performed for surgical planning, including sentinel lymph node mapping to rule out other lymph node involvement. An aggressive lesion, consistent with soft tissue of the rostral right mandible was identified with intralesional contrast injection showing drainage of contrast within the right mandibular lymph nodes (Sentinel node). Based on the infiltrative and destructive nature reported in ameloblastic carcinoma in humans, a rostral mandibulectomy was performed, along with complete mandibular lymphadenectomy. The horse recovered uneventfully from surgery. In the early post‐operative period marked lymphoedema of the ventral mandibular region was observed, which resolved 4–5 days post‐operatively. Optimal cosmesis was maintained post‐operatively, and no recurrence has been observed to date – 12 months post‐surgery.
An stochastic multiattribute acceptability analysis‐based method for the multiattribute project portfolio selection problem with rank‐level information
Authors: Song Shiling , Ang Sheng , Yang Feng , Xia Qiong 
Published: June 2019

AbstractIn many cases of practical multiattribute project portfolio selection problems, it is hard to obtain accurate measurements of attributes and precise preference information. Even after a long and costly information gathering, the attribute measurements and the preference information can still be uncertain or inaccurate. Considerable cost saving will be obtained if the selection of an optimal project portfolio can be done using rank‐level information based on some or all the attributes, without knowing the preference information. In this paper, we propose a stochastic multiattribute acceptability analysis‐based method that can deal with mixed rank and cardinal attribute measurements and uses little or no weight information. In the proposed stochastic multiattribute acceptability analysis‐based method, the decision makers need not to express their preferences explicitly or implicitly, so it is particularly useful when no weight information is available at all. A numerical example involving selection of photovoltaic plants in an industrial province in Eastern China is provided to demonstrate the proposed method.
Transverse fatigue behaviour and residual stress analyses of double sided FSW aluminium alloy joints
Authors: Salvati Enrico , Everaerts Joris , Kageyama Koji , Korsunsky Alexander M. 
Published: June 2019

AbstractFriction stir welding (FSW) since its invention has been attracting relevant interest for joining aluminium alloys. Due to the nature of this process, the materials can be joint without melting. Thanks to this peculiar characteristic, the issues associated with the cooling from liquid phase are avoided or considerably reduced, such as cracking, porosity, and defects.However, as well as other well‐established welding techniques, the FSW process gives rise to formation of residual stress in the welding region and surrounding volume: heat and thermo‐mechanical affected zones. Presence of residual stress in a mechanical component is well‐known to affect its performance, particularly regarding fatigue at high number of cycles. Another aspect that influences the fatigue life is the underlying microstructure.In this work, we firstly study the residual stress field and the underlying microstructural features arising in FSW butt joints and their effect on the fatigue performance of this type of weldments. The evaluation of residual stress field is carried out by means of modern experimental techniques. In the first instance, synchrotron X‐ray powder diffraction was employed for two‐dimensional full field maps of residual stress. Corroboration of these measurements was done by exploiting the capability of focused ion beam and digital image correlation (FIB‐DIC), which is able to deliver pointwise absolute measurement of residual stress. A set of FSW samples were then tested under uniaxial fatigue loading at several loading ranges, in the high cycle fatigue regime, in order to understand whether the severity of loads affects the crack path and life endurance. Fractographic and electron backscattered diffraction (EBSD) analysis then revealed crack nucleation site and propagation mechanisms with the respect of the underlying microstructure. Outcome of these experimental studies is then thoroughly discussed.
Global community breaks at 60 m on mesophotic coral reefs
Authors: Lesser Michael P. , Slattery Marc , Laverick Jack H. , Macartney Keir J. , Bridge Tom C. 
Published: June 2019

AbstractAimMesophotic coral ecosystems (MCEs) are unique communities that support a high proportion of depth‐endemic species distinct from shallow‐water coral reefs. However, there is currently little consensus on the boundaries between shallow and mesophotic coral reefs and between upper versus lower MCEs because studies of these communities are often site specific. Here, we examine the ecological evidence for community breaks, defined here as species loss, in fish and benthic taxa between shallow reefs and MCEs globally.LocationGlobal MCEs.Time period1973–2017.Major taxa studiedMacrophytes, Porifera, Scleractinia, Hydrozoa, Octocorallia, Antipatharia and teleost fishes.MethodsWe used random‐effects models and breakpoint analyses on presence/absence data to identify regions of higher than expected species loss along a depth gradient of 1–69 m, based on a meta‐analysis of 26 studies spanning diverse photoautotrophic and heterotrophic taxa. We then investigated the extent to which points of high faunal turnover can be explained by environmental factors, including light, temperature and nutrient availability.ResultsWe found evidence for a community break, indicated by a significant loss of shallow‐water taxa, at ~ 60 m across several taxonomically and functionally diverse benthic groups and geographical regions. The breakpoint in benthic composition is best explained by decreasing light, which is correlated with the optical depths between 10 and 1% of surface irradiance. A concurrent shift in the availability of nutrients, both dissolved and particulate organic matter, and a shift from photoautotroph to heterotroph‐dominated assemblages also occurs at ~ 60 m depth.Main conclusionsWe found evidence for global community breaks across multiple benthic taxa at ~ 60 m depth, indicative of distinct community transitions between shallow and mesophotic coral ecosystems. Changes in the underwater light environment and the availability of trophic resources along the depth gradient are the most parsimonious explanations for the observed patterns.
Modified basic and specific (BASP) classification for pattern hair loss
Authors: Kim Beom Jun , Choi Jaewoong , Choe Sung Jay , Lee Solam , Lee Won‐Soo 
Published: June 2019

AbstractBackgroundIn 2007, Lee et al. introduced a basic and specific (BASP) classification for pattern hair loss that was comprehensive and applicable regardless of race or gender. However, this BASP classification has several limitations. Frontal type hair loss classification is relatively crude, and a specific hair loss pattern cannot be ascertained when hair loss is associated with the temporal and occipital areas.MethodsIn our modified BASP classification, frontal type classification was subdivided into five instead of three grades. Basic type classification remained the same as in the previous method. In addition, information regarding the involvement of the temporal or occipital scalp was recorded. Accuracy and ease of use were evaluated and compared with the existing BASP classification in 138 patients with pattern hair loss.ResultsTemporal or occipital involvement was observed in 14 patients, accounting for 11.1% of subjects. Final type accuracy was 82.5% in the existing BASP classification and 71.4% in the modified classification. Ease of use for two practitioners was 70.2 and 72.1% for the existing BASP classification, and 48.9 and 52.2% for the modified method.ConclusionWe expect that the modified BASP classification will overcome the limitations of the existing BASP classification. We believe this modified classification will be a valuable tool for pattern hair loss classification because of its classification of previously unclassified types.
Relationships of maternal body mass index and plasma biomarkers with childhood body mass index and adiposity at 6 years: The Children of SCOPE study
Authors: Dalrymple Kathryn V. , Thompson John M.D. , Begum Shahina , Godfrey Keith M. , Poston Lucilla , Seed Paul T. , McCowan Lesley M.E. , Wall Clare , Shelling Andrew , North Robyn , Cutfield Wayne S. , Mitchell Edwin A. ,   on behalf of the SCOPE Consortium
Published: June 2019

SummaryBackgroundMaternal obesity has been implicated in the origins of childhood obesity through a suboptimal environment in‐utero.ObjectiveWe examined relationships of maternal early pregnancy body mass index (BMI), overweight/obesity, and plasma biomarkers of obesity, inflammation, insulin resistance, and placental function with measures of childhood BMI and adiposity.MethodsBMIz‐score, sum of skinfold thicknesses (SST), body fat percentage (BFP, by bioelectrical impedance), and waist, arm, and hip circumferences were measured in 1173 6‐year‐old children of nulliparous pregnant women in the Screening for Pregnancy Endpoints (SCOPE) study, New Zealand. Relationships of maternal early pregnancy (15 weeks' gestation) BMI and biomarkers with these childhood anthropometric measures were assessed by linear regression, with appropriate adjustment.Results28.1% of mothers were classified as overweight and 10.1% with obesity; compared with normal weight mothers, the BFP of their children were 5.3% higher (0.16 SD [95% CI, 0.04‐0.29]p = .01) and 7.8% higher (0.27 [0.08‐0.47]p = .006) with comparable values for BMI z‐score and arm, waist, and hip circumferences. Early pregnancy maternal BMI and plasma placental growth factor (PlGF) were associated with higher child's SST, BMI z‐score, hip circumference, and BFP. None of the metabolic or inflammatory maternal biomarkers were associated with childhood obesity.ConclusionIn this contemporary large prospective cohort study with extensive maternal/childhood phenotyping and a high prevalence of maternal overweight/obesity, we found independent relationships of maternal early pregnancy BMI with childhood BMI and adiposity; similar associations were observed with PlGF, which may imply a role for placenta function in the developmental programming of childhood obesity risk.
Morphology and Phylogenetic Position of Two New Gregarine Species (Apicomplexa: Eugregarinorida) Parasitizing the Lubber Grasshopper Taeniopoda centurio (Drury, 1770) (Insecta: Orthoptera: Romaleidae) in Mexico
Authors: Medina‐Durán Jorge Humberto , Mayén‐Estrada Rosaura , Mariño‐Pérez Ricardo , Song Hojun 
Published: June 2019

AbstractEugregarines are understudied apicomplexan parasites of invertebrates inhabiting marine, freshwater, and terrestrial environments. Most currently known terrestrial eugregarines have been described parasitizing the gut from less than 1% of total insect diversity, with a high likelihood that the remaining insect species are infected. Eugregarine diversity in orthopterans (grasshoppers, locusts, katydids, and crickets) is still little known. We carried out a survey of the eugregarines parasitizing the Mexican lubber grasshopper, Taeniopoda centurio, an endemic species to the northwest of Mexico. We described two new eugregarine species from the gut of the host: Amoebogregarina taeniopoda n. sp. and Quadruspinospora mexicana n. sp. Both species are morphologically dissimilar in their life‐cycle stages. Our SSU rDNA phylogenetic analysis showed that both species are phylogenetically distant to each other, even though they parasitize the same host. Amoebogregarina taeniopoda n. sp. clustered within the clade Gregarinoidea, being closely related to Amoebogregarina nigra from the grasshopper Melanoplus differentialis. Quadruspinospora mexicana n. sp. clustered within the clade Actinocephaloidea and grouped with Prismatospora evansi, a parasite from dragonfly naiads. Amoebogregarina taeniopoda n. sp. and Q. mexicana n. sp. represent the first record of eugregarines found to infect a species of the family Romaleidae.
Lunar influences in the diet habits and selectivity of larval clingfish Gobiesox marmoratus
Authors: De Los Ríos‐Méndez Juanita , Kanamori Gabriela A. , Landaeta Mauricio F. 
Published: June 2019

AbstractThe influence of the lunar cycle on the feeding habits, feeding success and selectivity was tested in situ on larval stages of the clingfish Gobiesox marmoratus (Gobiesocidae). Gobiesox marmoratus larvae and their prey were collected in the water column during the lunar cycle in austral spring 2015 and 2016, in shallow waters (<30 m depth) of El Quisco, central Chile. Feeding incidence was high (80–100%) throughout the moon cycle. The diet was composed of 32 prey items, being gastropod larvae and invertebrate eggs the most important. Prey composition varied among lunar phases with the lowest prey richness during new moon. During 2016, G. marmoratus larvae ingested the lowest number, but the largest prey during new moon. In spring 2015, there were no significant changes in the abundance of each prey taxon in the water column among moon phases, but in spring 2016 there were significant differences in the abundance of cypris and copepod nauplii, particularly between full and new moon. Feeding selectivity index showed that gastropod larvae were positively selected throughout the lunar cycle during spring 2015. In spring 2016, invertebrate eggs were selectively ingested in full moon and third quarter, but at a new moon G. marmoratus larvae selected gastropod larvae. Mean temperature of the water column and its vertical gradient and nocturnal cloud cover influences the feeding success of larval G. marmoratus. Lunar illumination favoured only an increase of richness of prey items. Therefore, nocturnal cloud cover precludes a greater influence of lunar illumination in the larval trophic ecology of this crypto‐benthic fish.
Disaster cosmologies in comparative perspective: Islam, climate change and the 2010 floods in Pakistan's Southern Punjab
Authors: Ahmad Ali Nobil 
Published: June 2019

AbstractAs humanity enters a new era of climate‐induced unpredictability, research into the role of religion in shaping perceptions of, and responses to disaster will become increasingly important. This is particularly true of South Asia, which contains dense populations certain to be adversely affected by climate change. This contribution explores the way religion shapes and mediates responses to disaster in Pakistan. Where previous work in this field has focused on extremists and militants, mine considers currents of lived Islam that take explicit stances on questions of natural resource development. Drawing upon extensive primary data, I identify two distinct disaster cosmologies permeating state and society. First, I consider the official Islam of experts and policymakers, whose approach to development is derived from, but arguably surpasses the modernism of British and American colonial and Cold War paradigms in its dogmatic, faith‐based belief in the imperative of mastering and exploiting nature. The second is an altogether contrasting formation embedded in a political protest movement representing a marginalized constituency, the Siraiki speaking population of Southern Punjab, which mobilized flood affectees in the aftermath of the 2010 floods around issues of social and environmental justice.