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.

Development of appropriateness explicit criteria for cataract extraction by phacoemulsification

Background: Consensus development techniques were used in the late 1980s to create explicit criteria for the appropriateness of cataract extraction. We developed a new appropriateness of indications tool for cataract following the RAND method. We tested the validity of our panel results. Methods: Criteria were developed using a modified Delphi panel judgment process. A panel of 12 ophthalmologists was assembled. Ratings were analyzed regarding the level of agreement among panelists. We studied the influence of all variables on the final panel score using linear and logistic regression models. The explicit criteria developed were summarized by classification and regression tree analysis. Results: Of the 765 indications evaluated by the main panel in the second round, 32.9% were found appropriate, 30.1% uncertain, and 37% inappropriate. Agreement was found in 53% of the indications and disagreement in 0.9%. Seven variables were considered to create the indications and divided into three groups: simple cataract, with diabetic retinopathy, or with other ocular pathologies. The preoperative visual acuity in the cataractous eye and visual function were the variables that best explained the panel scoring. The panel results were synthesized and presented in three decision trees. Misclassification error in the decision trees, as compared with the panel original criteria, was 5.3%. Conclusion: The parameters tested showed acceptable validity for an evaluation tool. These results support the use of this indication algorithm as a screening tool for assessing the appropriateness of cataract extraction in field studies and for the development of practice guidelines.

Dramatické texty přeložené a cenzurované ve frankistickém Španělsku (šedesátá léta): divadlo a film jako kontrolovaná představení

Texto en checo. Editora de la obra Petra Vavroušová.

The Spanish corporate structure through interlocking directorates

[EN] The aim of this paper is to determine to what extent globalization pressures are changing interlocking directorate networks modeled on continental capitalism into Anglo-Saxon models. For this purpose we analyse the Spanish network of interlocks, comparing the present structure (2012) with that of 1993 and 2006. We show how, although Spanish corporative structure continues to display characteristics of the continental economies, some major banks are significantly reducing industrial activity. Nevertheless, the financial organizations continue to maintain a close relationship with sectors such as construction and services. The analysis of the network of directorates shows a retreat in activity of industrial banking in Spain. Two large Spanish financial institutions, BSCH and La Caixa, continue to undertake activities of industrial banking in 2006, but this activity is significantly reduced in 2012. According to the theories on the role of the interlocking directorates, the companies in these sectors assure their access to banking credit by incorporating advisors from financial organizations to their board of directors. We cannot conclude that the structure of the Spanish corporate network has become a new case of Anglo-Saxon structure, but we got indications that are becoming less hierarchic as banks seem to slowly abandon centrality positions. This is especially salient if we compare the networks of 2006 and 2012, which show a continuing decrease of the role of banks and insurance companies in the network.

Paleoambiente y antropización en los Pirineos de Navarra durante el Holoceno medio (VI-IV PDF milenios cal. BC): Una perspectiva palinológica

Homenaje a Ignacio Barandiarán Maestu / coord. por Javier Fernández Eraso, Juan Santos Yanguas