Behavioral profile of adults with Prader-Willi syndrome : correlations with individual and environmental variables

Background: Maladaptive behavior has been reported as a phenotypical feature in Prader–Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. Methods: The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Results: Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Conclusions: Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS.

Víctimas del terrorismo residentes en la CAPV. Desazón y esperanza en víctimas indirectas de asesinatos. Año 2013

La investigación, encomendada y financiada por la Dirección de Víctimas y Derechos Humanos del Gobierno Vasco (DVDH), se centra en una parcela de la victimización indirecta provocada por el terrorismo. El objetivo principal, ante un posible final del terrorismo, consiste en sondear, a título individual, las percepciones sobre la situación personal y social en 2013, de un grupo significativo de familiares, residentes en dicha comunidad, de personas asesinadas por grupos terroristas desde 1960. Se trata de conocer mediante una investigación científica, con el mayor rigor posible, su opinión sobre la evolución en el trato recibido por parte de diferentes agentes sociales, con énfasis en la administración de justicia penal y penitenciaria, y, en particular, analizar sus actitudes sobre el debate público en torno a ellas y sobre su contribución social. La consecución del objetivo principal permitirá alcanzar otros más específicos en relación con la identificación de los intereses prioritarios de las propias víctimas en futuras políticas y actividades victimales. De esta manera, se facilitará la reflexión sobre los elementos que provocan procesos de victimización secundaria, de cara a evitarlos o aminorarlos, así como los factores que facilitan a las víctimas sus procesos de recuperación, entendidos en un sentido complejo, amplio y diverso.