Reward-allocation judgments in Romania: a factorial survey approach

We investigated reward-allocation judgments when positive outcomes (monetary rewards) were distributed and the allocator was not a co-recipient, in a sample of 200 Romanian students. Within a full factorial survey design, seven factors, selected to affect the allocation decision, were orthogonally varied. The factors reflect individual characteristics of the recipients (gender, age, contribution, need, work experience) and situational characteristics (future work interaction and task routineness). Romanian students preferred to allocate rewards applying an equity-based distribution model. Work experience and task routineness were also significant, yet less important. The recipient gender has no effect on allocation judgments. In terms of between-respondents variability, we found that students' gender, work experience, and age do not have an impact on reward-allocation judgments. We discuss implications of our findings for organizations and practitioners

Histological study of human periodontal tissue following biologically oriented preparation technique (BOPT)

The aim of this study was to conduct histological analysis of a human tooth resected with the periodontal insertion apparatus intact following treatment using biologically oriented preparation technique (BOPT). This descriptive histological dento-periodontal study used an anterior tooth extracted with the surrounding periodontal tissues intact, following prosthetic restoration with BOPT. The sample patient was recruited from among those attending the Department of Dental Medicine at the Faculty of Medicine and Dentistry, University of Valencia (Spain). Eight serial sections of the restored tooth were processed. The relative location and histological characteristics of the cemented prosthetic crown, the dental tissues of the tooth prepared by BOPT technique, and the periodontal tissues were analyzed. Structural analysis of the neoformed junctional epithelium showed that the number of layers decrease apically until there was a single row of cells perfectly adhered to the acellular cementum, and beneath the epithelium a connective tissue evidently free from inflammation. The tissues of the neoformed periodontium (gingival ligament, sulcular epithelium, junctional epithelium) presented histologic normality. Biologically oriented preparation technique is a reliable alternative to conventional horizontal finish lines

Acquiescent and Socially Desirable Response Styles in Cross-Cultural Value Surveys

The present chapter presents two studies examining the differential effects of acquiescence and social desirability on value scores across cultures. In the first study, culture-level acquiescence indexes were extracted from data in eight multinational surveys, and culture-level social desirability scores were obtained from a meta-analysis of the Marlowe-Crowne Social Desirability Scale. Both types of indexes were correlated with cultural value dimensions reported in the literature and with indicators of affluence. We found that affluence explains a substantial proportion of the variance in the association of response styles with value scores in all the surveys. The second study investigated effects of score standardization. This study was based on a large cross-cultural data set collected with the Schwartz Value Survey (SVS). We found that value score standardization had some effect on the correlations of acquiescence with various value types, but only limited effects on social desirability. We conclude that affluence affects the relationship of response styles and value scores. Implications for the interpretation of cross-cultural differences in response styles and value surveys are discussed

Consenso Delphi sobre la transición de pediatría a adulto en las consultas de epilepsia

Delphi; Neuropediatría; TransiciónDelphi; Paediatric neurology; TransitionDelphi; Neuropediatria; TransicióIntroduction The transition from paediatric to adult neurology consultations in patients with epilepsy must be understood as a process in which the patient acquires independence in the management of their disease and faces new issues related to adulthood. The aim of this study is to gather the opinion of experts in epilepsy on this transition in order to issue recommendations. Methods Using a Delphi consensus process, 54 paediatric and adult neurologists with expertise in epilepsy expressed their degree of agreement on 38 statements on about the transition from paediatric to adult consultations, grouped into the following sections: (1) preparation and moments prior to transition; (2) transition process; and (3) moments after the transition and follow-up. Results After 2 rounds, consensus was reached for 33 statements (86.8%). For the remaining 5 statements (13.2%), there was neither agreement nor disagreement. Among the subjects for which consensus was strongest was the involvement of the patient and family, who must be provided sufficient information to resolve their doubts and concerns. Before referral, the clinical report must be reviewed to ensure that it contains all relevant information on the disease. During the first follow-up visits after the transition, the patient's knowledge should be brought up to date in order to promote their autonomy. Conclusion The clinical report, early preparation, and complete case review are considered fundamental in the process of transition from paediatric to adult neurology care.Introducción La transición desde neuropediatría a las consultas de neurología para adultos en pacientes con epilepsia ha de entenderse como un proceso en el que el paciente adquiere una independencia en el manejo de la enfermedad y afronta nuevos aspectos relacionados con la edad adulta. El objetivo de este estudio es conocer la opinión de expertos en epilepsia sobre esta transición para poder ofrecer recomendaciones. Métodos Mediante un consenso Delphi, 54 neuropediatras y neurólogos expertos en epilepsia mostraron su grado de acuerdo en 38 aseveraciones sobre la transición de pediatría a la consulta de adultos agrupadas en los siguientes bloques: 1) preparación y momento previo a la transición; 2) durante la transición; y 3) momento posterior a la transición y seguimiento. Resultados Tras dos rondas, se alcanzó un consenso en el acuerdo en 33 aseveraciones (86,8%). En las 5 aseveraciones restantes (13,2%) no hubo ni acuerdo ni desacuerdo. Entre los acuerdos más importantes destaca la implicación del paciente y la familia a los que es necesario aportar información suficiente para disipar sus dudas y miedos. Antes de la derivación, se ha de comprobar que el informe clínico contiene toda la información relevante de la enfermedad. Durante las primeras visitas de seguimiento tras la transición, se han de actualizar los conocimientos de los pacientes para lograr su autonomía. Conclusión El informe clínico, la preparación anticipada y la revisión completa del caso se consideran fundamentales en el proceso de transición de pediatría a la consulta de adultos.EISAI Farmacéutica SA funded this project, without participating in any way in study design, data analysis, or drafting of the article. EISAI Farmacéutica SA also funded the assistance with manuscript drafting

Pathogenic convergence of CNVs in genes functionally associated to a severe neuromotor developmental delay syndrome.

Background Complex developmental encephalopathy syndromes might be the consequence of unknown genetic alterations that are likely to contribute to the full neurological phenotype as a consequence of pathogenic gene combinations. Methods To identify the additional genetic contribution to the neurological phenotype, we studied as a test case a boy, with a KCNQ2 exon-7 partial duplication, by single-nucleotide polymorphism (SNP) microarray to detect copy-number variations (CNVs). Results The proband presented a cerebral palsy like syndrome with a severe motor and developmental encephalopathy. The SNP array analysis detected in the proband several de novo CNVs, nine partial gene losses (LRRC55, PCDH9, NALCN, RYR3, ELAVL2, CDH13, ATP1A2, SLC17A5, ANO3), and two partial gene duplications (PCDH19, EFNA5). The biological functions of these genes are associated with ion channels such as calcium, chloride, sodium, and potassium with several membrane proteins implicated in neural cell-cell interactions, synaptic transmission, and axon guidance. Pathogenically, these functions can be associated to cerebral palsy, seizures, dystonia, epileptic crisis, and motor neuron dysfunction, all present in the patient. Conclusions Severe motor and developmental encephalopathy syndromes of unknown origin can be the result of a phenotypic convergence by combination of several genetic alterations in genes whose physiological function contributes to the neurological pathogenic mechanism.post-print783 K

Agenesis of the corpus callosum in a newborn with Turner mosaicism

The agenesis of the corpus callosum results from a failure in the development of the largest fiber bundle that connects cerebral hemispheres. Patient’s outcome is influenced by etiology and associated central nervous system malformations. We describe a child with Turner syndrome (TS) mosaicism, with particular phenotype features and a complete agenesis of the corpus callosum. To our knowledge, this is the second case report of TS mosaicism associated with complete agenesis of the corpus callosum. Anatomical brain magnetic resonance imaging and diffusion tensor imaging were useful to confirm the complete absence of the corpus callosum, evaluate associated central nervous system malformations, visualize abnormal white matter tracts (Probst bundles) and assess the remaining commissures

Methodology of a Natural History Study of a Rare Neurodevelopmental Disorder: Alternating Hemiplegia of Childhood as a Prototype Disease

Here, we describe the process of development of the methodology for an international multicenter natural history study of alternating hemiplegia of childhood as a prototype disease for rare neurodevelopmental disorders. We describe a systematic multistep approach in which we first identified the relevant questions about alternating hemiplegia of childhood natural history and expected challenges. Then, based on our experience with alternating hemiplegia of childhood and on pragmatic literature searches, we identified solutions to determine appropriate methods to address these questions. Specifically, these solutions included development and standardization of alternating hemiplegia of childhood-specific spell video-library, spell calendars, adoption of tailored methodologies for prospective measurement of nonparoxysmal and paroxysmal manifestations, unified data collection protocols, centralized data platform, adoption of specialized analysis methods including, among others, Cohen kappa, interclass correlation coefficient, linear mixed effects models, principal component, propensity score, and ambidirectional analyses. Similar approaches can, potentially, benefit in the study of other rare pediatric neurodevelopmental disorders

An Indigenous Measure of Social Desirability Across Non-Western Countries

Cross-cultural differences in Social Desirability (SD) could be partly due to the nonequivalence of constructs, items, or other challenges of cross-cultural research. We tested to what extent a Mexican, indigenous scale of SD, capturing both positive and negative features of SD, would be useful in other countries. Data were collected in convenience samples in eight countries (Argentina, China, Colombia, Costa Rica, Lebanon, Mexico, Nicaragua, and Spain) in order to test the psychometric accuracy and invariance of the factor structure. Values of Tucker’s factor congruence coefficients (gauging invariance) and tests of the similarity of the cross-country similarity of Cronbach’s alpha (gauging internal consistency) revealed that SD, as measured by this indigenous list, is stable and comparable across cultures. The results are interpreted in a conceptual framework in which SD is viewed as a culturally embedded communication style that people use to integrate successfully into their groups

Investigación sobre la producción vitivinícola en el bajo valle del Ebro (ss. VII-I a. C.): un proyecto interdisciplinar de arqueología experimental

We present the results of an interdisciplinary project aimed at the experimental reproduction of wine making processes (production, storage, and conservation) according to historical-archaeological data from Iron Age (7th-1st centuries BC) north-eastern Iberia. The experiment has documented an annual cycle, from grape harvesting, production of must, its transformation into wine and the subsequent conservation of the resulting drink. In order to check its progress, its quality and suitability for consumption, the alcoholic degree, total acidity, pH, volatile acidity, colour, and potassium concentration were analysed and evaluated. In addition, quarterly wine tastings were conducted to assess its organoleptic quality and degree of taste acceptability. A basic microbiological control of the main yeasts and bacteria identified during the process was carried out, too. The results provide fundamental data on the stages of wine production process and preservation conditions, both of which are key to analysing the role of wine consumption in the social practices of Iron Age communities in the north-east of the Iberian Peninsula.Se presentan los resultados de un proyecto interdisciplinar que ha tenido como objetivo la reproducción experimental de los procesos de producción, almacenamiento y conservación del vino de acuerdo a los datos histórico-arqueológicos procedentes del nordeste peninsular durante la Edad del Hierro (ss. VII-I a. C.). El experimento ha documentado el ciclo anual, desde la recolección de la uva, la elaboración del mosto y su transformación en vino, hasta la posterior conservación de la bebida resultante. Con el fin de comprobar su evolución, calidad y aptitud para el consumo, se han analizado y valorado el grado alcohólico, la acidez total, el pH, la acidez volátil, el color y la concentración de potasio. Asimismo se han realizado catas trimestrales para determinar la calidad organoléptica y su grado de aceptabilidad gustativa. Paralelamente, se ha efectuado un control microbiológico básico de las principales levaduras y bacterias localizadas durante el proceso. Los resultados aportan datos relevantes sobre las fases del proceso productivo y las condiciones de conservación del vino, ambas claves para analizar el papel de su consumo en la práctica social de las comunidades de la Edad del Hierro del noreste peninsular

Phosphomannomutase deficiency (PMM2-CDG): Ataxia and cerebellar assessment

Background: Phosphomannomutase deficiency (PMM2-CDG) is the most frequent congenital disorder of glycosylation. The cerebellum is nearly always affected in PMM2-CDG patients, a cerebellar atrophy progression is observed, and cerebellar dysfunction is their main daily functional limitation. Different therapeutic agents are under development, and clinical evaluation of drug candidates will require a standardized score of cerebellar dysfunction. We aim to assess the validity of the International Cooperative Ataxia Rating Scale (ICARS) in children and adolescents with genetically confirmed PMM2-CDG deficiency. We compare ICARS results with the Nijmegen Pediatric CDG Rating Scale (NPCRS), neuroimaging, intelligence quotient (IQ) and molecular data. Methods: Our observational study included 13 PMM2-CDG patients and 21 control subjects. Ethical permissions and informed consents were obtained. Three independent child neurologists rated PMM2-CDG patients and control subjects using the ICARS. A single clinician administered the NPCRS. All patients underwent brain MRI, and the relative diameter of the midsagittal vermis was measured. Psychometric evaluations were available in six patients. The Mann-Whitney U test was used to compare ICARS between patients and controls. To evaluate inter-observer agreement in patients' ICARS ratings, intraclass correlation coefficients (ICC) were calculated. ICARS internal consistency was evaluated using Cronbach's alpha. Spearman's rank correlation coefficient test was used to correlate ICARS with NPCRS, midsagittal vermis relative diameter and IQ. Results: ICARS and ICARS subscores differed between patients and controls (p < 0.001). Interobserver agreement of ICARS was "almost perfect" (ICC = 0.99), with a "good" internal reliability (Cronbach's alpha = 0.72). ICARS was significantly correlated with the total NPCRS score (rs 0.90, p < 0.001). However, there was no agreement regarding categories of severity. Regarding neuroimaging, inverse correlations between ICARS and midsagittal vermis relative diameter (rs -0.85, p = 0.003) and IQ (rs -0.94, p = 0.005) were found. Patients bearing p.E93A, p.C241S or p.R162W mutations presented a milder phenotype. Conclusions: ICARS is a reliable instrument for assessment of PMM2-CDG patients, without significant inter-rater variability. Despite our limited sample size, the results show a good correlation between functional cerebellar assessment, IQ and neuroimagingFor the first a correlation between ICARS, neuroimaging and IQ in PMM2-CDG patients has been demonstratedThe work was supported by national grants PI14/00021, PI11/01096, PI11/01250, and PI10/00455 from the National Plan on I+D+I, cofinanced by ISC-III (Subdirección General de Evaluación y Fomento de la Investigación Sanitaria) and FEDER (Fondo Europeo de Desarrollo Regional) and IPT-2012- 0561-010000 from MINECO. Three research groups (U-746, U-737 and U703) from the Centre for Biomedical Research on Rare Diseases (CIBER-ER), Instituto de Salud Carlos III, Spain, have worked together for the present stud