Personal Factors, Living Environments, and Specialized Supports: Their Role in the Self-Determination of People with Intellectual Disability.

The self-determination of people with disabilities, and specifically people with intellectual disabilities (ID), is a growing issue due to its relevance in the field of inclusion and human rights. Although research has shown a significant relationship between self-determination and intelligence, other factors also contribute to its development. The purpose of this study was to understand what other variables may be influencing self-determination. Using the scores from 483 adolescents and adults with ID who completed the AUTODDIS scale, we performed inferential and regression analyses to determine the relationships between levels of self-determination, personal variables (sex, age, severity of ID), and contextual variables (living environment, specialized supports). We found that self-determination is affected by the severity of ID, and when this variable is controlled for, greater self-determination is mainly related to receiving occupational support and support for autonomy and independent living. Results also showed that, together with ID severity, occupational and psychoeducational support, as well as support for autonomy and independent living, were also predictors of the level of self-determination. In conclusion, this study confirms the importance of contextual variables in the development of self-determination in people with ID, placing the focus of intervention on social opportunities.

Assessment of self-determination in mental health: A new application field of the AUTODDIS scale.

OBJECTIVE: Self-determination enhances well-being and quality of life. It is also considered a fundamental pillar in improving the efficacy of interventions used to treat people with severe mental disorders (SMD). Yet the assessment of self-determination in relation to mental health requires further research. The aim of this study was to analyze the adequacy and psychometric properties of the AUTODDIS scale in a Spanish population with SMD. METHOD: The scale was originally developed and validated to assess self-determination in people with intellectual disability. The scale was administered to a sample of 333 adults with SMD (M = 47.6 years, SD = 11.68), most of whom were receiving treatment in an outpatient setting or at long-stay care facilities in six specialized centers in Spain. RESULTS: The quality of the items and the reliability of the scale and its subscales were analyzed. External validity was also explored, and confirmatory factor analysis was used to analyze the fit of the data to different models. The results indicate that the scale demonstrated sufficient evidence of reliability and validity, supporting the adequacy of its use in the field of mental health. CONCLUSIONS AND IMPLICATIONS FOR PRACTICE: The use of this scale as a tool to assess self-determination and its domains in the mental health field is warranted. The article also discusses the need for more research and assessment tools to facilitate decision making by clinical and organizational stakeholders in promoting self-determination. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Incidence and Prevalence of Children's Diffuse Lung Disease in Spain.

BACKGROUND: Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. METHODS: Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. RESULTS: A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). CONCLUSIONS: We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Incidence and Prevalence of Children's Diffuse Lung Disease in Spain / Incidencia y prevalencia de las neumopatías intersticiales pediátricas en España

Background Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. Methods Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. Results A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28–10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81–51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2–18 years of age compared with children 0–2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). Conclusions We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Antecedentes Las neumopatías intersticiales pediátricas, también conocidas con el acrónimo chILD (del inglés children's Interstitial Lung Diseases), es un grupo heterogéneo de enfermedades raras con morbimortalidad relevante, cuyo diagnóstico y clasificación son complejos. Los estudios epidemiológicos son escasos. El objetivo de este trabajo fue analizar la incidencia y la prevalencia de chILD en España. Métodos Estudio prospectivo observacional multicéntrico en pacientes de 0 a 18 años afectos de chILD para analizar la incidencia y la prevalencia en España, a partir de datos recogidos en 2018 y 2019. Resultados Se recogieron 381 casos de chILD entre 51 unidades de neumología pediátrica de toda España, que cubrían el 91,7% de la población pediátrica. La incidencia promedio fue 8,18 (IC 95%: 6,28-10,48) casos nuevos/millón de niños por año. La prevalencia promedio fue de 46,53 (IC 95%: 41,81-51,62) casos/millón de niños. El grupo de edad con mayor prevalencia fue el de niños menores de un año. Se observaron diferentes entidades en niños de 2 a 18 años en comparación con niños de 0 a 2 años. Los diagnósticos más frecuentes fueron: glucogenosis intersticial pulmonar primaria en neonatos (17/65), hiperplasia de células neuroendocrinas en lactantes de uno a 12 meses (44/144), hemosiderosis pulmonar idiopática en niños de uno a 5 años (13/74), neumonía por hipersensibilidad en niños de 5 a 10 años (9/51) y esclerodermia en mayores de 10 años (8/47). Conclusiones Encontramos una mayor incidencia y prevalencia de chILD que las descritas previamente, probablemente debido a un mayor conocimiento y detección de estas enfermedades raras.

Deep Phenotyping and Genetic Characterization of a Cohort of 70 Individuals With 5p Minus Syndrome.

Chromosome-5p minus syndrome (5p-Sd, OMIM #123450) formerly known as Cri du Chat syndrome results from the loss of genetic material at the distal region of the short arm of chromosome 5. It is a neurodevelopmental disorder of genetic cause. So far, about 400 patients have been reported worldwide. Individuals affected by this syndrome have large phenotypic heterogeneity. However, a specific phenotype has emerged including global developmental delay, microcephaly, delayed speech, some dysmorphic features, and a characteristic and monochromatic high-pitch voice, resembling a cat's cry. We here describe a cohort of 70 patients with clinical features of 5p- Sd characterized by means of deep phenotyping, SNP arrays, and other genetic approaches. Individuals have a great clinical and molecular heterogeneity, which can be partially explained by the existence of additional significant genomic rearrangements in around 39% of cases. Thus, our data showed significant statistical differences between subpopulations (simple 5p deletions versus 5p deletions plus additional rearrangements) of the cohort. We also determined significant "functional" differences between male and female individuals.

Training women's pelvic floor muscles during pregnancy and postpartum at primary health centers: a best practice implementation project.

INTRODUCTION AND AIMS: Pelvic floor muscle training (PFMT) enhances muscle strength and coordination, and prevents urinary incontinence associated with giving birth. The goal was to improve women's PFMT during pregnancy and postpartum in La Rioja, Spain. METHODS: This evidence implementation project used the JBI evidence implementation framework. First, we conducted the PFMT baseline audit: to assess the criteria for midwife training, we sent a questionnaire to all midwives. To measure the criteria for PFMT and urinary incontinence risk assessment, we reviewed the medical records of 60 women selected through consecutive sampling and 15 other women at risk of urinary tract infections (UTIs) for the inclusion criteria in a PFMT program. Second, we used the Getting Research into Practice guidelines to identify barriers to the fulfillment of each criterion and design and implement strategies to improve compliance. Finally, we repeated the audit to measure compliance and verify the changes. RESULTS: The midwives received updated PFMT, optimizing compliance with the first criterion from 67 to 100%. The remaining criteria -- providing PFMT for pregnant women, identifying women at high risk of incontinence, and facilitating a supervised PFMT program for women at high risk of UTIs -- went from 0% compliance to 17, 96, and 67%, respectively. CONCLUSION: This project was effective in building consensus, improving midwives' practice, and facilitating mothers' PFMT. The project continued during the novel coronavirus [coronavirus disease 2019 (COVID-19)] epidemic in Spain as the midwives, women, and managers were involved in the change because of a well designed registration system and the use of online tools for communication between midwives and women.

Self-Determination in People with Intellectual Disability: The Mediating Role of Opportunities.

The Convention on the Rights of Persons with Disabilities have proclaimed the basic right of people to make one's own choices, have an effective participation and inclusion. Research in the field of disability have stressed self-determination as a key construct because of its impact on their quality of life and the achievement of desired educational and adulthood related outcomes. Self-determination development must be promoted through specific strategies and especially, by providing tailored opportunities to practice those skills. Providing these opportunities across environments could be especially relevant as a facilitator of self-determination development. This manuscript aims to ascertain if opportunities at home and in the community to engage in self-determined actions are mediating the relationship between people intellectual disability level and their self-determination. Results have confirmed direct effects of intellectual disability level on self-determination scores. Indirect effects also predicted self-determination and almost all its related components (self-initiation, self-direction, self-regulation, self-realization, and empowerment) through opportunities in the community and at home. Autonomy was predicted by the intellectual disability level through an indirect effect of opportunities at home, but not in the community. These results highlight the need for further research to better operationalize and promote contextually rooted opportunities for people with intellectual disability to become more self-determined.

Infusión subcutánea continua de insulina en menores de 6 años: evolución a largo plazo / Continuous subcutaneous insulin infusion in children less than 6 years-old: long-term progress

Objetivos: Valorar la eficacia y la seguridad del tratamiento con infusión subcutánea continua de insulina (ISCI) en niños menores de 6 años durante periodos prolongados de tiempo y evaluar si alcanzan criterios de adecuado control glucémico. Métodos: Estudio retrospectivo de 27 niños que iniciaron tratamiento con ISCI entre 2003-2014. Edad de inicio de ISCI: 4 años (2,9-4,7); 56% varones. Se recogen: edad de inicio de diabetes, tiempo de evolución de diabetes, HbA1c (HPLC Menarini, valor normal 5,1±0,31%), dosis insulina (u/kg/día), número de controles de glucosa capilar/día, número de tramos basales/día, porcentaje de insulina basal, ratios insulina/ración hidratos carbono (I/HC), episodios de hipoglucemia grave y de CAD (episodios/100 pacientes-año), porcentajes de normoglucemia (70-180mg/dl), hiperglucemia (> 180mg/dl) e hipoglucemia (<70mg/dl), glucemia media, desviación estándar y coeficiente variación ([desviación estándar/glucemia media]×100). Análisis estadístico por SPSS. Resultados: La HbA1c disminuye de 6,9%(6,7-7,5) a 6,8%(6,4-7,1) el primer año, posteriormente se mantiene <6,8% durante el periodo de seguimiento (mediana 5 años [3-6]). Antes de ISCI el 74% tenían HbA1c <7,5% y al año el 96%. Media de controles de glucosa capilar/día de 10(9-11). No se observaron cambios significativos en la dosis de insulina. Hubo un episodio de CAD y un episodio de hipoglucemia grave durante el seguimiento. La ratio I/HC al desayuno fue superior a la de otras ingestas (0,92u/r vs. 0,55, 0,6 y 0,5 en comida, merienda y cena). Conclusiones: El tratamiento con ISCI es eficaz y seguro en menores de 6 años durante periodos prolongados de tiempo. Permite alcanzar los objetivos de buen control metabólico recomendados por la Asociación Americana de Diabetes y la Sociedad Internacional de Diabetes Pediátrica y del Adolescente, sin incremento de efectos adversos (AU)

Objective: The aims of the study are to evaluate the efficacy and safety of continuous subcutaneous insulin infusion (CSII) treatment in pre-school children with type I diabetes, and to assess whether the criteria of good metabolic control are achieved. Method: A review was performed on the medical charts of patient's < 6 years of age who started CSII treatment between 2003 and 2014. The cohort consisted of 27 patients (mean age 4 (2.9-4.7) years, 56% males). An analysis was made including the age at onset, type I diabetes duration, HbA1c (HPLC, Menarini, normal value 5.1 ± 0.31%), insulin dose (u/kg/day), number of capillary blood glucose measurements, number of baseline processes per day, % baseline/total insulin (B/TI), insulin ratios (I/HC) at different meals, severe hypoglycaemia (HS episodes/100 patients years), DKA events, percentages of normal blood glucose (70-180mg/dl), hyperglycaemia (>180mg/dl), and hypoglycaemia (<70 mg/dl), mean blood glucose, standard deviation and coefficient of variation (SD/mean glucose ×100). Statistical analysis was performed using SPSS. Results: HbA1c decreased from 6.9% (6.7-7.5) to 6.8% (6.4-7.1) after one year of CSII. Afterwards, it remained under 6.8% during the follow-up (median 5 years [3-6]). Prior to CSII, 74% of children had HbA1c levels < 7.5%. It increased to 96% after one year of CSII. Median blood glucose measurements /day was 10 (9-11). Total insulin dose did not change significantly. During the follow-up, there was one episode of DKA and one episode of HS. I/HC at breakfast were higher than at other meals (0.92 vs. 0.55, 0.6 and 0.5, respectively). Conclusions: CSII is effective and safe in pre-school children. It allows good metabolic control (based on Society for Paediatric and Adolescent Diabetes / American Diabetes Association criteria) to be achieved and maintained for long periods of time without an increase in adverse events (AU)

[Continuous subcutaneous insulin infusion in children less than 6 years-old: long-term progress]. / Infusión subcutánea continua de insulina en menores de 6 años: evolución a largo plazo.

OBJECTIVE: The aims of the study are to evaluate the efficacy and safety of continuous subcutaneous insulin infusion (CSII) treatment in pre-school children with type I diabetes, and to assess whether the criteria of good metabolic control are achieved. METHOD: A review was performed on the medical charts of patient's<6 years of age who started CSII treatment between 2003 and 2014. The cohort consisted of 27 patients (mean age 4 (2.9-4.7) years, 56% males). An analysis was made including the age at onset, type I diabetes duration, HbA1c (HPLC, Menarini, normal value 5.1±0.31%), insulin dose (u/kg/day), number of capillary blood glucose measurements, number of baseline processes per day, % baseline/total insulin (B/TI), insulin ratios (I/HC) at different meals, severe hypoglycaemia (HS episodes/100 patients years), DKA events, percentages of normal blood glucose (70-180mg/dl), hyperglycaemia (>180mg/dl), and hypoglycaemia (<70mg/dl), mean blood glucose, standard deviation and coefficient of variation (SD/mean glucose ×100). Statistical analysis was performed using SPSS. RESULTS: HbA1c decreased from 6.9% (6.7-7.5) to 6.8% (6.4-7.1) after one year of CSII. Afterwards, it remained under 6.8% during the follow-up (median 5 years [3-6]). Prior to CSII, 74% of children had HbA1c levels < 7.5%. It increased to 96% after one year of CSII. Median blood glucose measurements /day was 10 (9-11). Total insulin dose did not change significantly. During the follow-up, there was one episode of DKA and one episode of HS. I/HC at breakfast were higher than at other meals (0.92 vs. 0.55, 0.6 and 0.5, respectively). CONCLUSIONS: CSII is effective and safe in pre-school children. It allows good metabolic control (based on Society for Paediatric and Adolescent Diabetes / American Diabetes Association criteria) to be achieved and maintained for long periods of time without an increase in adverse events.

Entrenamiento para la mejora de disfunciones atencionales en niños y adolescentes con Síndrome de Asperger a través de estimulación cognitiva directa / Training to improve attentional dysfunction in children and adolescents with Asperger Syndrome through direct cognitive stimulation

Esta investigación analiza la capacidad de generalización del aprendizaje en niños y adolescentes con Síndrome de Asperger a aspectos cognitivos atencionales con demandas diferentes a las ejercitadas mediante la utilización de un programa de estimulación cognitiva directa. La muestra estuvo constituida por 15 casos con Síndrome de Asperger entre 7 y 15 años (M = 12; DT = 2.7) con dificultades atencionales, medidas a través de instrumentos de orientación psicométrica, a los que se aplicó un programa de entrenamiento en atención dos horas semanales durante seis meses consecutivos. Los resultados del análisis pretest-postest mostraron diferencias estadísticamente significativas en todos los subsistemas atencionales. En atención selectiva las diferencias son de magnitud moderada en las variables relacionadas con la velocidad de procesamiento, en mantenida el tiempo de reacción disminuyó considerablemente (magnitud de la diferencia moderada) y aumentaron los aciertos disminuyendo los errores (ambos con una magnitud de diferencia grande). En atención alternante se observó un mayor número de aciertos, y en dividida se obtuvieron mayores niveles de respuestas totales (los dos de magnitud moderada). Se concluye que el rendimiento atencional en los casos analizados mejora tras la aplicación de un programa de entrenamiento cognitivo específico siendo capaces de generalizar el aprendizaje a medidas psicométricas

This research analyzes the generalization ability of learning in children and adolescents with Asperger Syndrome to attentional cognitive aspects with different demands on exercised through the use of a direct cognitive stimulation program. The sample consisted of 15 cases with Asperger between 7 and 15 years (M = 12, SD = 2.7) with attentional difficulties, measured by psychometric orientation instruments, to which an attention training program was implemented to two hours per week for six consecutive months. The results of the pretest-posttest analysis showed statistically significant differences in all attentional subsystems. Selective attention differences are moderate in variables related to processing speed, in sustained attention the reaction time significantly decreased (moderate magnitude of the difference) and increased the successes reducing errors (both with a large magnitude difference) . In alternating attention, a greater number of successes were observed and in split attention higher levels of total responses (both moderate magnitude) were obtained. It is concluded that the attentional performance in the cases analyzed improvement after applying a cognitive training program specifically being able to generalize learning psychometric measures

[Voiding symptoms changes after vaginal sling surgery for female stress urinary incontinence]. / Cambios en la sintomatología miccional de la incontinencia urinaria de esfuerzo tras la cirugía con técnicas de cabestrillo transvaginal.

OBJECTIVES: To evaluate the changes in voiding symptoms in female patients with stress urinary incontinence (SUI) undergoing transvaginal sling techniques over a five-year period in the Department of Urology at the University Hospital of Albacete. METHODS: Between November 2001 and December 2005 126 patients with SUI (mean age 57.09 years; 36-78) underwent transvaginal sling techniques (Sling in Fast, TVK TOT). All patients were evaluated clinically and urodynamically. RESULTS: Average body mass index (BMI) was 28.14 kg/m2 (SD 4.66; 95% CI: 27.32-28.96). 92 patients (73%) presented between 2-4 previous pregnancies. 99 patients (80.9%) have had birth labour between 2 and 4 times. All of them were vaginal birth labours except 12 cases (9.5%) in which caesarean section had been performed. Daytime voiding frequency after surgery was over 120 minutes in 112 patients (88.9%). Night-time voiding frequency was equal or less than twice in 110 patients (87.3%). 104 patients (82.5%) presented at least two leaking episodes per day, and 105 patients (83.3%) needed to wear one pad per day or less during the last week before follow-up visit. The number of urinary leak episodes per day diminished in 114 patients (90.5%) with a mean decrease of 9.65 episodes (95% CI: 8.56-10.79) (p < 0.0001). Ninety-four patients (76.4%) were completely dry. CONCLUSIONS: Development of new surgical techniques for the treatment of SUI have improved results and diminished the number of complications, an expression of which is the favourable evolution of voiding changes after surgery.

Cambios en la sintomatología miccional de la incontinencia urinaria de esfuerzo tras la cirugía con técnicas de cabestrillo transvaginal / Voiding symptoms changes after vaginal sling surgery for female stress urinary incontinence

Objetivo: Evaluar los cambios en la sintomatología miccional de las pacientes con incontinencia urinaria de esfuerzo (IUE), intervenidas con técnicas de cabestrillo transvaginal, durante un periodo de cinco años en el Servicio de Urología del Complejo Hospitalario Universitario de Albacete (CHUA). Método: Entre noviembre de 2001 y diciembre de 2005, se han intervenido en nuestro centro a 126 pacientes (36-78; media 57,09 años) afectas de IUE, mediante técnicas de cabestrillo transvaginal (Técnicas de Sling In Fast, TVT y TOT). Todas las pacientes fueron evaluadas mediante clínica y urodinamia. Resultados: El índice de masa corporal (IMC) medio fue de 28,14 Kg/m2 (DE: 4,66; IC 95%: 27,32-28,96). 92 pacientes (73%) presentaban entre 2 y 4 embarazos. 99 pacientes (80,9%) presentaban entre 2 y 4 partos. Todos los partos fueron vaginales a excepción de 12 casos (9,5%) en que se practicó cesárea. Tras la cirugía la frecuencia miccional diurna fue superior a 120 minutos en 112 pacientes (88,9%), con respecto a la nocturna 110 pacientes (87,3%), se levantaban por la noche a orinar menos de 2 veces. 104 pacientes (82,5%) presentaban menos de 2 episodios de escape a día y 105 pacientes (83,3%) necesitaban como mucho usar una compresa al día durante la última semana tras la cirugía. Disminuyó en número de escapes al día tras la cirugía en 114 pacientes (90,5%), con una disminución media de 9,65 escapes (IC: 95%; 8,56-10,79) (p<0,0001). 94 pacientes (76,4%) estaban totalmente secas. Conclusiones: El desarrollo de nuevas técnicas quirúrgicas para el tratamiento de la IUE, ha favorecido los resultados y ha disminuido el número de complicaciones, manifestándose en la evolución favorable de los cambios miccionales tras la cirugía (AU)

Objectives: To evaluate the changes in voiding symptoms in female patients with stress urinary incontinence (SUI) undergoing transvaginal sling techniques over a five-year period in the Department of Urology at the University Hospital of Albacete. Methods: Between November 2001 and December 2005 126 patients with SUI (mean age 57.09 years; 36-78) underwent transvaginal sling techniques (Sling in Fast, TVT, TOT). All patients were evaluated clinically and urodynamically. Results: Average body mass index (BMI) was 28.14 kg/m2 (SD 4.66; 95% CI: 27.32-28.96). 92 patients (73%) presented between 2-4 previous pregnancies. 99 patients (80.9%) have had birth labour between 2 and 4 times. All of them were vaginal birth labours except 12 cases (9.5%) in which caesarean section had been performed. Daytime voiding frequency after surgery was over 120 minutes in 112 patients (88.9%). Night-time voiding frequency was equal or less than twice in 110 patients (87.3%). 104 patients (82.5%) presented at least two leaking episodes per day, and 105 patients (83.3 %) needed to wear one pad per day or less during the last week before follow-up visit. The number of urinary leak episodes per day diminished in 114 patients (90.5 %) with a mean decrease of 9.65 episodes ( 95 % CI : 8.56-10.79 ) (p < 0 . 0001 ). Ninety-four patients (76.4 % ) were completely dry. Conclusions : Development of new surgical techniques for the treatment of SUI have improved results and diminished the number of complications , an expression of which is the favourable evolution of voiding changes after surgery (AU)