bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gender disorders in learning disability – a systematic review
Authors: Wood Ellena , Halder Neel 
Published: October 2014

Purpose
– The purpose of this paper is to systematically review the literature on gender identity disorder (GID) and associated gender disorders in people with learning disabilities and autism, specifically focusing on aetiology, treatment and management.

Design/methodology/approach
– This study reviewed all the published papers about individuals with both a learning disability and/or autistic spectrum disorder and a gender disorder. Papers from 1980 onwards were included as this was the year of the introduction of GID to the ICD-10. Gender disorders were taken to include the following: GID, transsexualism, cross-dressing, transvestitism or a gender-related sexual disorder.

Findings
– In total, 16 papers described 43 individuals meeting the inclusion criteria. There was a dearth of guidance on appropriate treatment or management.

Research limitations/implications
– Only English language papers were searched. This review points towards more research needed in this area.

Originality/value
– In collating relevant papers the review begins the search for evidence regarding aetiology, treatment and management of gender disorders in an area where evidence-based guidelines are needed.
Early intervention for children with learning disabilities: making use of what we know
Authors: Gore Nick , Hastings Richard , Brady Serena 
Published: October 2014

Purpose– The purpose of this paper is to present a rationale for increasing initiatives for early intervention of emotional and behaviour difficulties.Design/methodology/approach– The authors draw on existing literature regarding rates of emotional and behavioural difficulties together with risk factors and processes related to the development of such difficulties.Findings– Rates of emotional and behavioural difficulties amongst children with learning and developmental difficulties are high. A combination of factors relating to the child, the family system, and wider social contexts is likely to account for this.Research limitations/implications– Increased attempts to provide early intervention to children with learning and developmental disabilities together with their families are warranted. Recommendations are made regarding how the development of such supports might best be taken forward.Originality/value– Whilst drawing on pre-existing literature, the value of this paper is the way in which this has been drawn together to provide an overview of risk and development of behavioural and other difficulties amongst children with learning/developmental disabilities.
Four‐year natural clinical course of pulmonary epithelioid hemangioendothelioma without therapy
Authors: Haro Akira , Saitoh Genkichi , Tamiya Sadafumi , Nagashima Akira 
Published: October 2014

AbstractPulmonary epithelial hemangioendothelioma is a rare low to intermediate malignant vascular tumor originating from vascular endothelial cells. The therapy for this disease, if possible, is surgical resection. However, there is no standard treatment for patients with multiple unresectable lesions. We present the case of a 42‐year‐old woman treated with a natural clinical course of hemangioendothelioma for four years without therapy. The nodules have increased in number and size extremely slowly, and the patient is alive and asymptomatic four years after diagnosis.
The Effects of Abnormal Executive Pay Dispersion: Evidence from Firm Performance and Executive Turnover
Authors: Byun Sanghyuk 
Published: October 2014

AbstractThis study examines the relation between pay dispersion among top executives and firm performance and executive turnover. I find that deviations from normal dispersion are associated with deterioration in firm performance and higher executive turnover. I also document that a positive relation between abnormal pay dispersion and executive turnover is driven by low‐ranked executives. This study contributes to the existing literature by suggesting that abnormal pay dispersion is an important factor that influences managerial behavior in addition to raw pay dispersion.
Microvascular Pathology and Morphometrics of Sporadic and Hereditary Small Vessel Diseases of the Brain
Authors: Craggs Lucinda J.L. , Yamamoto Yumi , Deramecourt Vincent , Kalaria Raj N. 
Published: October 2014

AbstractSmall vessel diseases (SVDs) of the brain are likely to become increasingly common in tandem with the rise in the aging population. In recent years, neuroimaging and pathological studies have informed on the pathogenesis of sporadic SVD and several single gene (monogenic) disorders predisposing to subcortical strokes and diffuse white matter disease. However, one of the limitations toward studying SVD lies in the lack of consistent assessment criteria and lesion burden for both clinical and pathological measures. Arteriolosclerosis and diffuse white matter changes are the hallmark features of both sporadic and hereditary SVDs. The pathogenesis of the arteriopathy is the key to understanding the differential progression of disease in various SVDs. Remarkably, quantification of microvascular abnormalities in sporadic and hereditary SVDs has shown that qualitatively the processes involved in arteriolar degeneration are largely similar in sporadic SVD compared with hereditary disorders such as cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Important significant regional differences in lesion location within the brain may enable one to distinguish SVDs, where frontal lobe involvement appears consistently with almost every SVD, but others bear specific pathologies in other lobes, such as the temporal pole in CADASIL and the pons in pontine autosomal dominant microangiopathy and leukoencephalopathy or PADMAL. Additionally, degenerative changes in the vascular smooth muscle cells, the cerebral endothelium and the basal lamina are often rapid and more aggressive in genetic disorders. Further quantification of other microvascular elements and even neuronal cells is needed to fully characterize SVD pathogenesis and to differentiate the usefulness of vascular interventions and treatments on the resulting pathology.
CADASIL and CARASIL
Authors: Tikka Saara , Baumann Marc , Siitonen Maija , Pasanen Petra , Pöyhönen Minna , Myllykangas Liisa , Viitanen Matti , Fukutake Toshio , Cognat Emmanuel , Joutel Anne , Kalimo Hannu 
Published: October 2014

AbstractCADASIL and CARASIL are hereditary small vessel diseases leading to vascular dementia. CADASIL commonly begins with migraine followed by minor strokes in mid‐adulthood. Dominantly inherited CADASIL is caused by mutations (n > 230) in NOTCH3 gene, which encodes Notch3 receptor expressed in vascular smooth muscle cells (VSMC). Notch3 extracellular domain (N3ECD) accumulates in arterial walls followed by VSMC degeneration and subsequent fibrosis and stenosis of arterioles, predominantly in cerebral white matter, where characteristic ischemic MRI changes and lacunar infarcts emerge. The likely pathogenesis of CADASIL is toxic gain of function related to mutation‐induced unpaired cysteine in N3ECD. Definite diagnosis is made by molecular genetics but is also possible by electron microscopic demonstration of pathognomonic granular osmiophilic material at VSMCs or by positive immunohistochemistry for N3ECD in dermal arteries.In rare, recessively inherited CARASIL the clinical picture and white matter changes are similar as in CADASIL, but cognitive decline begins earlier. In addition, gait disturbance, low back pain and alopecia are characteristic features. CARASIL is caused by mutations (presently n = 10) in high‐temperature requirement. A serine peptidase 1 (HTRA1) gene, which result in reduced function of HTRA1 as repressor of transforming growth factor‐β (TGF β) ‐signaling. Cerebral arteries show loss of VSMCs and marked hyalinosis, but not stenosis.
Combined maxillary sinus floor elevation and endonasal endoscopic sinus surgery for coexisting inflammatory sinonasal pathologies: a one‐stage double‐team procedure
Authors: Abu‐Ghanem Sara , Kleinman Shlomi , Horowitz Gilad , Balaban Sagi , Reiser Vadim , Koren Ilan 
Published: October 2014

AbstractObjectivesTo report our experience with combined one‐stage double‐team maxillary sinus floor elevation (SFE) and endonasal endoscopic sinus surgery (ESS) procedure for concomitant inflammatory sinonasal pathologies.Material and methodsClinical records of all patients that underwent maxillary SFE in conjunction with endonasal ESS for the treatment of inflammatory sinonasal pathologies between 2011 and 2013 were retrospectively reviewed. All included patients had a sinonasal‐related pathology that was first suggested by the referring physician and was later confirmed clinically and radiographically by our combined team comprised of otorhinolaryngologist and maxillofacial surgeons.ResultsFifteen combined SFE+ESS surgeries were performed using either xenograft–allograft mixture or autograft–xenograft–allograft mixture. The study group included seven males and eight females, whose median age was 55 years (range, 45–78 years). Seven patients underwent a unilateral SFE, and eight patients underwent bilateral SFEs. During the same session, four patients also underwent septoplasty for deviated nasal septum, five patients underwent bilateral maxillary antrostomy, 10 patients underwent unilateral maxillary antrostomy, and six patients underwent maxillary sinus cyst resection. Seven combined procedures were performed under active infection. There were no intra‐operative complications, and all SFE+ESS combined procedures were successful. Three patients required extended postoperative antibiotic treatment for persistent sinusitis. One patient reported infraorbital hypoesthesia.ConclusionsWe first report the promising outcomes of the double‐team one‐stage SFE+ESS procedure performed by a combined team of otorhinolaryngologist and maxillofacial surgeons, including on patients presenting with an infection of the sinuses at the time of surgery.
Increased permeability of the epithelium of middle ear cholesteatoma
Authors: Koizumi H. , Suzuki H. , Ohbuchi T. , Kitamura T. , Hashida K. , Nakamura M. 
Published: October 2014

ObjectiveWe investigated the electrical impedance of and the expressions of tight junction molecules in the cholesteatoma epithelium to provide supporting evidence for the acid lysis theory of bone resorption in middle ear cholesteatoma.MethodsStudy subjects were patients with primary acquired middle ear cholesteatoma and those with non‐cholesteatomatous chronic otitis media who underwent tympanomastoidectomy. The electrical impedance of the cholesteatoma epithelium was measured during tympanomastoidectomy by loading alternating currents of 320 Hz and 30.7 kHz. The expressions of tricellulin (MARVELD2), claudin‐1 (CLDN1) and claudin‐3 (CLDN3) were examined by fluorescence immunohistochemistry and quantitative reverse transcription‐polymerase chain reaction.ResultsThe electrical impedance of the cholesteatoma epithelium was significantly lower than that of the post‐auricular skin and external auditory canal skin at both 320 Hz and 30.7 kHz. Immunoreactivity for MARVELD2, CLDN1 and CLDN3 was localised mainly in the granular layer, and to lesser degree, in the horny and spinous layers in both the cholesteatoma tissue and post‐auricular skin. Fluorescence intensity was moderate for MARVELD2, weak for CLDN1 and strong for CLDN3. The expressions of MARVELD2, CLDN1 and CLDN3 mRNA were significantly lower in the cholesteatoma tissue than in the post‐auricular skin.ConclusionsThese results indicate the increased permeability of the cholesteatoma epithelium and suggest that this change is, at least partially, dependent on the decrease in the expressions of the tight junction molecules. This evidence supports the acid lysis hypothesis of bone resorption in cholesteatoma.
The Effect of Applying Alternate IPCC Climate Scenarios to Marine Reserve Design for Range Changing Species
Authors: Makino Azusa , Klein Carissa J. , Possingham Hugh P. , Yamano Hiroya , Yara Yumiko , Ariga Toshinori , Matsuhasi Keisuke , Beger Maria 
Published: October 2014

AbstractEffectively protecting of biodiversity in the future relies on reserves that accommodate potential climate change impacts. Climate predictions are based on plausible ranges of greenhouse gas concentration scenarios from the IPCC, called Representative Concentration Pathways (RCPs). It is unknown how different scenarios influence spatial prioritization, particularly for species that change their range due to climate change. Using corals in Japan, we explore differences in priorities under three RCPs (RCP8.5, 4.5, and 2.6), comparing three time frames (current conditions, near future, and distant future). We targeted three temperature zones representing different coral community types, determined from predictions of sea‐surface temperature for three RCPs. Results showed that using one RCP prediction to design a reserve system does a poor job at meeting conservation targets for other RCPs, missing up to 100% of the targets. We emphasize the importance of focusing conservation investment in “no regrets” areas that are important under every RCP.
Imaging of the major salivary glands
Authors: Afzelius Pia , Nielsen Ming‐Yuan , Ewertsen Caroline , Bloch Klaus Poulsen 
Published: October 2014

SummaryThe major salivary glands, submandibular, parotid and sublingual glands play an important role in preserving the oral cavity and dental health. Patients with problems of the major salivary glands may present with symptoms such as dry mouth, dysphagia and obstruction of duct, inflammation, severe dental caries or swelling. Imaging plays an important role in visualization of morphology and function, to establish a diagnosis, for treatment, and for surgical planning. There are several options for diagnostic imaging: plain radiography, sialography, ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT), salivary gland scintigraphy and 18F‐FDG positron emission tomography (PET). We present an overview of the modalities in relation to common salivary gland disease.
Reliability of health‐related physical fitness tests in adolescents: the MOVE Program
Authors: Vanhelst Jérémy , Béghin Laurent , Fardy Paul S. , Ulmer Zekya , Czaplicki Grégory 
Published: October 2014

SummaryThe aim was to examine the reliability of health‐related physical fitness tests that were used in a French health promotion program ‘Move…A priority for your health’. Participants were 174 French youth (88 children and 86 adolescents) aged 8·2–16·2 years. Aerobic fitness, muscular strength, speed and flexibility were tested using 20‐m shuttle run test, ½ mile run test, basketball throw, standing long jump, shoulder stretch and 20/30/50‐m sprint tests. Reliability was calculated for the basketball throw, standing long jump, shoulder stretch and sprint tests. The tests were performed two times, 1 week apart on the same day of the week. Reliability was examined with intraclass correlation coefficients (ICCs) and Bland–Altman analysis. With the exception of the ½ mile run test, which resulted in moderate agreement (0·66), all tests had high reliability. ICCs were 0·97, 0·93, 0·91 and 0·93 for the sprint test, basketball throw, shoulder stretch and the standing long jump, respectively. The differences obtained between the first and the second trial were non‐significant. Results from this study indicate that the BOUGE health‐related physical fitness battery, administrated by physical education teachers, was reliable for measuring health‐related components of fitness in children and adolescents in a school setting.
Can the assessment of ABCB1 gene expression predict its function in vitro?
Authors: Kosztyu Petr , Dolezel Petr , Vaclavikova Radka , Mlejnek Petr 
Published: October 2014

AbstractIncreased expression of the ABCB1 gene in cancer cells is usually connected with occurrence of multidrug resistance (MDR) and poor prognosis. However, the correlation between ABCB1 expression and MDR phenotype is difficult to prove in clinical samples. Most of the researchers believe that these difficulties are due to the poor reliability and sensitivity of assays for detection of ABCB1 expression in clinical samples. However, the complexity of P‐gp mediated resistance cannot be reduced to the methodical difficulties only. Here, we addressed the question how widely used methods for detection of ABCB1 expression levels could predict its functional activity and thus its contribution to drug resistance in defined conditions in vitro. The ABCB1 expression was assessed at the mRNA level by quantitative real‐time polymerase chain reaction (qRT‐PCR), and at the protein level by flow cytometry using UIC2 antibody. The ABCB1 function was monitored using a calcein AM accumulation assay. We observed that K562 cells have approximately 320 times higher level of ABCB1 mRNA than HL‐60 cells without detectable function. In addition, resistant K562/Dox cells exhibited significantly higher ABCB1 mRNA expression than resistant K562/HHT cells. However, the functional tests clearly indicated opposite results. Flow cytometric assessment of P‐gp, although suggested as a reliable method, contradicted the functional test in K562/Dox and K562/HHT cells. We further used a set of MDR cells expressing various levels of P‐gp. Similarly here, flow cytometry not always corresponded to the functional analysis. Our results strongly suggest that an approach which exclusively relies on a simple correlation between ABCB1 expression, either at the mRNA level or protein level, and overall resistance may fail to predict actual contribution of P‐gp to overall resistance as the data indicating transporter expression reflect its function only roughly even in well‐defined in vitro conditions.
Predicting the water retention characteristics of UK mineral soils
Authors: Hollis J. M. , Lilly A. , Higgins A. , Jones R. J. A. , Keay C. A. , Bellamy P. 
Published: October 2014

SummaryThe management of water resources in compliance with regulatory requirements increasingly uses national environmental impact assessments incorporating models of soil solute transport. Most such models use either continuous functions designed to solve the Richards equation, or capacity‐type functions based on water contents between specified soil water pressure heads. The work described here has used three separate sets of measured water‐release data representative of the whole of the UK to develop a set of pedotransfer functions (PTFs) for predicting volumetric water contents at seven pressure heads on the water release curve ranging from 0 to −1500 kPa. A theoretically‐based structural stratification of the data was proposed and tested. Within each grouping, multiple regression analysis was used to derive equations for predicting the water contents at each pressure head, based on a selected set of predictors. The resulting PTFs gave a significantly better prediction than PTFs derived from the unstratified data and also than the widely used HYPRES PTFs. For all non‐sandy horizons not subject to regular cultivation, additional PTFs incorporating a climatic variable, the average annual potential soil moisture deficit (PSMD) gave a further improvement in prediction in drier areas of the UK with PSMD greater than 130 mm. Mean errors associated with using the PTFs to parameterize continuous function models and a capacity model for calculating water availability for cereals (APcereals) are ±16.4 and 16.9%, respectively.
Deficiency of brain structural sub‐network underlying post‐ischaemic stroke apathy
Authors: Yang S. , Hua P. , Shang X. , Cui Z. , Zhong S. , Gong G. , William Humphreys G. 
Published: October 2014

Background and purposeThis study aimed to reveal the structural basis of post‐ischaemic stroke apathy, especially in relation to disruptions in structural connectivity.MethodsEighty‐eight participants were included. The Apathy Evaluation Scale, clinician version, was used to characterize the severity of apathy. Diffusion tensor imaging tractography was used to examine white matter integrity and to reconstruct white matter networks using 90 nodes based on the automated anatomical labeling atlas. The degree for each node was extracted to determine the relationship to the severity of apathy.ResultsApathy was not significantly associated with damage to any single brain region. The degrees of 24 nodes (limbic system, three nodes; frontal lobe, six; basal ganglia, two; temporal lobe, three; parietal lobe, three; insula, two; occipital lobe, five) were significantly correlated to the Apathy Evaluation Scale scores. These 24 nodes constituted an apathy‐related sub‐network and its global and local efficiencies were negatively correlated with apathy levels (global, r = −0.54, P < 0.01; local, r = −0.64, P < 0.01). Multivariate logistic regression indicated that decreased global efficiency of this sub‐network was an independent risk factor for apathy (odds ratio 0.03, 95% confidence interval 0.01–0.04, P = 0.007). Efficiencies of the non‐apathy‐related sub‐network (the remaining 66 nodes) did not correlate or predict the presence of apathy.ConclusionsPost‐stroke apathy is not due to the dysfunction of a single region or circuit. Rather, it results from disconnection of a complex sub‐network of brain regions. This provides new insights into the neuroanatomical basis of post‐stroke apathy.
Anatomical localization of isocitrate dehydrogenase 1 mutation: a voxel‐based radiographic study of 146 low‐grade gliomas
Authors: Wang Y. , Zhang T. , Li S. , Fan X. , Ma J. , Wang L. , Jiang T. 
Published: October 2014

Background and purposeA brain tumor's location is associated with the genetic profile of its tumor precursor cells. Mutations in isocitrate dehydrogenase 1 (IDH1) are an early event in tumor development and play a critical role in gliomagenesis. This study was conducted to specify the anatomical characteristics of IDH1 mutation in low‐grade gliomas and to further explore the origin of gliomas with IDH1 mutation. The impact of IDH1 mutation on disease prognosis was also evaluated.MethodsThe pre‐operative magnetic resonance images obtained from 146 patients with histologically confirmed low‐grade glioma were analyzed retrospectively. All tumors were manually marked and registered to the standard location. Voxel‐based lesion‐symptom mapping analysis was used to identify brain regions associated with a high occurrence of IDH1 mutation. Progression‐free survival and overall survival were estimated using the Kaplan–Meier method, and potential prognostic factors were evaluated using the multivariate proportional hazards model.ResultsIsocitrate dehydrogenase 1 mutated low‐grade gliomas occurred most frequently in the frontal lobe, and specifically in the area surrounding the rostral extension of the lateral ventricles. Additionally, it was demonstrated that IDH1 mutation was an independent predictor for longer progression‐free survival and overall survival.ConclusionsLow‐grade gliomas with IDH1 mutation are region‐specific and preferentially located surrounding the rostral extension of the lateral ventricles. Furthermore, such mutations are associated with a favorable clinical outcome.