Impacto sociobiomédico de la sobreprotección: el Síndrome de Wolfram como ejemplo paradigmático / Impacto sociobiomédico da superproteção: a síndrome de Wolfram como um exemplo paradigmático / Sociobiomedical impact of overprotection: Wolfram syndrome as a paradigmatic example

The aim of this article is to analyze the possible existence of overprotection, its socialimpact, and its effects on the quality of life of people with Wolfram syndrome. The research261Cultura de los Cuidados. 1º Cuatrimestre 2022. Año XXVI. nº 62method is mix: quantitative and qualitative. The quantitative study is based on a correlationaldescriptive observational design. The sample consisted of 31 patients aged between 15 and 46years, diagnosed with Wolfram syndrome in Spain. The quality of life of the patients was analyzedthrough a questionnaire and the social behaviors of overprotection through a structured ad-hocinterview. On the other hand, a qualitative questionnaire on risk perception and social uncertaintywas made. The social uncertainty to which families are subjected leads them to sometimes takeoverprotective behaviors. However, our results show that this happens more in the group ofpatients without partners, as well as in the scale absence of overload and free time. The group ofpeople with a partner has greater social support, less overprotection, greaterphysical/psychological well-being, absence of work overload/leisure time and overall quality oflife.

El objetivo de este artículo es analizar la posible existencia de sobreprotección y analizarsu impacto social y sus efectos en la calidad de vida de personas con Síndrome de Wolfram. Lametodología de investigación es mixta: cuantitativa y cualitativa. El estudio cuantitativo se basaen un diseño observacional correlacional-descriptivo. En él, la muestra estuvo compuesta por 31pacientes españoles con Síndrome de Wolfram con edades comprendidas entre los 15 y 46 años.Se analizó la calidad de vida de los pacientes por medio de un cuestionario y las conductas socialesde sobreprotección por medio de una entrevista estructurada ad-hoc. Por otro lado, se realizó uncuestionario cualitativo sobre la percepción del riesgo y la incertidumbre social. La incertidumbresocial a la que se ven sometidas las familias les conduce a tomar, en ocasiones, comportamientosde sobreprotección. Nuestros resultados muestran que esto sucede más en el grupo de pacientessin pareja, asimismo en la escala de ausencia también se produce una sobrecarga y tiempo libre.El grupo de personas con pareja presentan un mayor apoyo social, menor sobreprotección, mayorbienestar físico/psíquico, ausencia de sobrecarga laboral/tiempo libre y calidad de vida global.

O objetivo deste artigo é analisar a possível existência de superproteção e analisar seuimpacto social e seus efeitos na qualidade de vida de pessoas com Síndrome de Wolfram. Ométodo de pesquisa é quantitativo e qualitativo. O estudo quantitativo é baseado em um desenhoobservacional descritivo-correlacional. A amostra foi composta por 31 pacientes espanhóis comSíndrome de Wolfram com idades entre 15 e 46 anos. A qualidade de vida dos pacientes foianalisada por meio de questionário e os comportamentos sociais superprotetores por meio deentrevista estruturada ad hoc. Por outro lado, foi realizado um questionário qualitativo sobre apercepção de risco e incerteza social. A incerteza social a que estão submetidas as famílias levaas a assumir, por vezes, comportamentos superprotetores. Nossos resultados mostram que issoacontece mais no grupo de pacientes sem companheiro, da mesma forma que na escala deausência também ocorre sobrecarga e tempo livre. O grupo de pessoas com companheiro262Cultura de los Cuidados. 1º Cuatrimestre 2022. Año XXVI. nº 62apresenta maior suporte social, menor superproteção, maior bem-estar físico / mental, ausênciade sobrecarga de trabalho / tempo livre e qualidade de vida geral.

Promoting Emotional Well-being in Hospitalized Children and Adolescents With Virtual Reality: Usability and Acceptability of a Randomized Controlled Trial.

The aim of this study was to describe and compare small-sized preliminary data on the usability and acceptability of a randomized controlled trial. This study compares a one-to-one cognitive-behavioral treatment and a virtual reality treatment for children and adolescents hospitalized for long-term physical conditions. The final sample was composed of 19 children and adolescents with chronic illness who were hospitalized. The sample was randomly allocated to two intervention groups: cognitive-behavioral usual treatment and virtual reality-based treatment. Participants in the virtual reality group demonstrated higher perceived efficacy scores for every treatment component than for traditional treatment. Statistically significant differences were found for the total usability and acceptability scores. Participants in the virtual reality group show high acceptability of all the virtual environments. The components and environments of virtual reality were well accepted. The virtual reality program received high scores in immersion, feasibility of use, and sense of reality. Technology-based treatments, that is, virtual reality, can provide motivational benefits in relation to traditional treatments.

Development and Validation of the EspaiJove.net Mental Health Literacy (EMHL) Test for Spanish Adolescents.

There is evidence of the effectiveness of implementing mental health literacy (MHL) programs. However, there are substantial limitations in the instruments available for measuring MHL. This study aimed to develop and validate the EspaiJove.net MHL test (EMHL) for Spanish adolescents by assessing its psychometric properties. The development of the EMHL test was conducted using item pool generation and a pilot study. A convenience sample of students aged 13-15 years (n = 355) participated in the validity study. Reliability was assessed for internal consistency and via test-retest. Convergent validity was evaluated by comparing the effect sizes among known groups with different levels of mental health knowledge, the correlation with mental health-related instruments, and the item discrimination index. A final version of a 35-item EMHL test was obtained with two parts: (i) a binary choice format (yes/no) for the identification of mental disorders; (ii) a multiple choice question with four possible answer options. Internal consistency was acceptable in the first part (Cronbach's alpha = 0.744; Guttman's lambda 2 = 0.773) and almost acceptable in the second part (Cronbach's alpha = 0.615; Guttman's lambda 2 = 0.643). The test-retest evaluation supported the stability of the test (first part, ICC = 0.578; second part, ICC = 0.422). No ceiling and floor effects were found. The EMHL test scores discriminated between known groups with different levels of mental health knowledge and it is associated with several-related constructs of MHL. Conclusions: The EMHL test is a relevant measure for assessing MHL in adolescents into Spanish context with acceptable validity and stability.

Natural history and clinical characteristics of 50 patients with Wolfram syndrome.

PURPOSE: To describe clinical characteristics of diabetes mellitus (DM) in a group of patients with Wolfram Syndrome (WS). METHODS: Descriptive, cross-sectional observational design. The sample consisted of 50 patients diagnosed with WS. Clinical criteria contributing to WS diagnosis were analyzed: diabetes mellitus (DM), optic nerve atrophy (OA), sensorineural deafness, urological and neurological dysfunction, among others. These parameters were assessed according to their presence/absence, age of onset, and various clinical-analytical parameters. RESULTS: All the patients studied presented DM and OA, with a mean age of onset of 5.4 ± .9 (1-14) years and 9 ± .9 (1-16) years, respectively. The remaining criteria were present with a variable frequency: 77% had diabetes insipidus, 66.7% auditory alterations, 77.8% neurogenic bladder, 61.1% neurological involvement, and 27.8% hypogonadism. A 16.7% of the patients had positive albuminuria (urinary albumin/creatinine ratio > 30 mg/g) and 72.2% had hyporreflexia. There were no significant differences in the age of diagnosis nor of the presence of different pathologies according to sex. CONCLUSIONS: The early presence of a non-autoimmune insulin dependent DM, should alert us of an "infrequent" diabetes syndrome. Wolfram's presumptive diagnosis could be established if juvenile-onset DM occurs concomitantly with OA, and this visual impairment is not attributable to diabetic retinopathy. Despite the long period of evolution of DM and altered values of HbA1c, the prevalence of microvascular complications in the sample are low.

Papel del control instruccional en el estudio de las tendencias de regulación verbal / Instructional Control Role in the Study of Verbal Regulatory Trends

La regulación verbal se ha estudiado ampliamente desde la teoría de los marcos relacionales por encontrarse en la base de múltiples fenómenos psicológicos, entre ellos, los procesos de aprendizaje en la infancia. El presente estudio tiene como objetivo principal evaluar si es más efectivo un procedimiento de aprendizaje con control instruccional (análogo experimental de la regulación verbal tipo pliance) versus un procedimiento sin control instruccional, esto es, sólo fortaleciendo las consecuencias directas (análogo experimental de la regulación verbal tipo tracking). Como objetivo secundario se pretende evaluar la efectividad de los tratamientos de manera diferencial según la tendencia inicial a seguir o no las instrucciones y evaluar su relación con variables implicadas en las diferencias individuales. Se utilizó un diseño de caso único A-B-C-A con réplicas y análisis entre sujetos. La muestra estuvo compuesta por nueve participantes (cinco chicos y cuatro chicas) con edades comprendidas entre 12 y 15 años. Los participantes realizaban una tarea de ordenador, en la que se evaluaba su tendencia a seguir o no las instrucciones, posteriormente realizaban dos bloques de entrenamiento, uno basado en el control instruccional y otro en moldeamiento por contingencias directas; finalmente se valoraba de nuevo su tendencia inicial. Adicionalmente, los participantes debían responder un cuestionario relativo a sus hábitos diarios. Los resultados mostraron que los participantes responden más rápido y con menor tasa de errores en el primer entrenamiento (control instruccional) y que variables como la rigidez, el autocontrol y el seguimiento instruccional podrían estar relacionadas con la facilidad en el aprendizaje de las mismas.

The Verbal regulation has been studied extensively from Relational Frame Theory for being at the basis of many psychological phenomena, including learning processes in childhood. The present study has as main objective to assess if more effective learning procedure with instructional control (experimental analogue type regulation pliance) vs. without instructional control procedure, only strengthening the direct (regulation similar type experimental tracking). The secondary aim is to: assess the effectiveness of treatments differentially according to the initial tendency to follow or not the instructions and evaluate its relationship with variables involved in individual differences. We used a single case design with replicas and analysis ABCA between subjects. The sample consisted of 9 participants (5 boys and 4 girls) aged between 12 and 15 years. Participants performed a computer task in which assessed their tendency to follow or not the instructions, then performed two blocks of training, one based on the control in shaping instructional and other direct contingencies; finally back valued their tendency initial. Additionally, participants had to answer a test on your daily habits. The results showed that participants respond faster and with less error rate in the first training (instructional control) and variables such as stiffness, self-control and instructional monitoring could be related to the ease in learning them.

Desarrollo de la personalidad: relación con sensibilidad a la ansiedad, rigidez y regulación verbal / Personality development: relationship with anxiety sensitivity, stiffness and regulation verbal

El objetivo de este estudio es analizar si existe relación entre sensibilidad a la ansiedad, perfeccionismo (patrón de rigidez vs. flexibilidad) y regulación verbal en niños con patrones disfuncionales de personalidad. La muestra estuvo compuesta de 123 participantes (53% chicas y 47% chicos), con edades entre 12 y 15 años, que en el momento del estudio se encontraban escolarizados. Se encontraron relaciones fuertes y moderadas entre las variables psicológicas medidas y las escalas de personalidad, así como con algunos síndromes clínicos. Específicamente se observa una clara relación entre la sensibilidad a la ansiedad y el perfeccionismo, así como una relación significativa entre la tendencia instruccional de los participantes y la variabilidad en las pruebas de personalidad. También se confirma la variable rigidez, como la que presenta mayor valor predictivo de la variabilidad en las pruebas de personalidad. Estos resultados pueden marcar algunas pautas para la creación de programas de prevención en los problemas de la personalidad disfuncional en población infantojuvenil

The aim of this study is to analyze the correlation between sensibility to the anxiety, perfectionism (rigidity vs. flexibility pattern), and verbal regulation in children with certain dysfunctional personality patterns. The sample consisted of 123 participants (53% girls and 47% boys), aged between 12 and 15 years at the time of the study, who were enrolled in two schools. Strong to moderate relationships between psychological variables and scales measures, as well as with clinical syndromes were found. Specifically a clear relation is observed between sensitivity to anxiety and perfectionism, as well as a significant relation between the instructional tendency of the participants and variability in the tests of personality. The study confirmed that the inflexibility variable presents the highest predictive value in the variability in personality tests. These results point toward some guidelines for the development of programs of prevention for problems of dysfunctional personality in children and youth population

Patrones de peroonalidad disfuncionales en niño y adolescentes: una revisión ffuncional - contextual / Dysfunctionl perrsonality patterns in children and adolescets: a functionaal - contextual review

Este trabajo pretende resaltar la importancia del estudio de los patrones de personalidad disfuncionales desde sus inicios al final de la infancia y principios de la adolescencia. Se revisará la evidencia empírica sobre los factores de riesgo de futuros estilos disfuncionales de personalidad en la etapa adulta y la posibilidad de que existan estos desórdenes como tal desde etapas muy tempranas. Se expondrá la visión de los trastornos de personalidad desde los manuales diagnósticos actuales, así como algunas particularidades del próximo DSM-V. Así mismo, se presenta un análisis del origen y desarrollo de dichos patrones disfuncionales de personalidad infanto-juveniles desde una visión funcional-contextual que incluirá el papel del lenguaje o la regulación verbal y del yo. Por último, se darán algunas perspectivas a tener en cuenta de cara a futuras investigaciones.

This paper aims to highlight the importance of the study of the dysfunctional patterns of personality from the beginning to the end of childhood and early adolescence. It will review the empirical evidence on the risk factors of future dysfunctional styles of personality in adulthood and the possibility of these disorders as such from very early stages. It will present the vision of personality disorders from current diagnostic manuals and some peculiarities of the future DSM-V. It also, presents an analysis of the origin and development of these dysfunctional patterns of infant-juvenile personality from a functional-contextual view including the role of language or the verbal regulation and the self. Finally, there are some perspectives to be considered for future research.

Transformación de funciones: Marcos de coordinación y oposición de acuerdo con equivalencia / Function transformation: Frames of coordination and opposition in equivalence relations

Esta investigación responde al interés por la dificultad que a veces tienen los sujetos para "cambiar" o "transformar" sus patrones de comportamiento. Investigaciones y teorías recientes desde el campo del análisis experimental del comportamiento y, más concretamente, desde una perspectiva de la Teoría de los Marcos Relacionales (RFT), abordan dicho fenómeno de una manera funcional y contextual. Desde este marco conceptual, el objetivo de este trabajo es establecer una historia experimental necesaria para generar repertorios de respuesta relacional de simetría y equivalencia en el marco de coordinación, y ver cómo son transformadas las funciones a un marco de oposición con base en las consecuencias que reciben los sujetos. Para ello, se diseñó y llevó a cabo un primer estudio cuyos resultados condujeron a variaciones en el procedimiento, lo que supuso la realización de un segundo experimento. La muestra estuvo compuesta de 16 sujetos, 7 en el primer experimento (5 mujeres y 2 hombres) y 9 en el segundo experimento (6 mujeres y 3 hombres) con edades comprendidas entre 20 y 40 años, quienes debían realizar una tarea de igualación a la muestra. Dicha tarea estuvo compuesta por dos fases y una evaluación final de las relaciones derivadas. La variable dependiente se definió como la ejecución del sujeto (número de ensayos) y la variable independiente como la introducción de imágenes aversivas y agradables en pro de la transformación de funciones. Finalmente, se discuten los datos intra e inter sujeto de los experimentos y algunas implicaciones prácticas.

This research is motivated by our interest in the difficulty that individuals show when trying to "change" or "convert" their behavioral patterns. Recent research and theories developed inside the experimental analysis of behavior, specifically from a Relational Framework Theory (RFT), expose that phenomenon from a functional-contextual way. From this conceptual frame, the purpose of this paper is to establish the experimental history needed to develop sets of symmetry relational responses and equivalence in the frames of sameness, and show the way the functions are transformed into a frame of opposition based upon the consequences that individuals receive. In order to achieve this goal, a first study was designed and carried out. The results obtained from this study led to several variations in the procedure and a second experiment was held. The sample was composed by 16 participants, ages between 20 and 40 who had to execute a matching-to-sample task. 7 individuals were used for the first experiment (5 females and 2 males) and 9 for the second (6 females and 3 males). The task was composed by two phases and a final evaluation of the derived relations. The dependent variable was defined as the execution of the individual (number of trials) and the independent variable as the introduction of aversive and pleasant pictures. Finally, the data obtained from both experiments is discussed intra e inter individual and some practical implications are outlined.