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.

Word frequency cues word order in adults: cross-linguistic evidence

[EN] One universal feature of human languages is the division between grammatical functors and content words. From a learnability point of view, functors might provide entry points or anchors into the syntactic structure of utterances due to their high frequency. Despite its potentially universal scope, this hypothesis has not yet been tested on typologically different languages and on populations of different ages. Here we report a corpus study and an artificial grammar learning experiment testing the anchoring hypothesis in Basque, Japanese, French, and Italian adults. We show that adults are sensitive to the distribution of functors in their native language and use them when learning new linguistic material. However, compared to infants’ performance on a similar task, adults exhibit a slightly different behavior, matching the frequency distributions of their native language more closely than infants do. This finding bears on the issue of the continuity of language learning mechanism.

Incidental Memory of Younger and Older Adults for Objects Encountered in a Real World Context

Effects of context on the perception of, and incidental memory for, real-world objects have predominantly been investigated in younger individuals, under conditions involving a single static viewpoint. We examined the effects of prior object context and object familiarity on both older and younger adults' incidental memory for real objects encountered while they traversed a conference room. Recognition memory for context-typical and context-atypical objects was compared with a third group of unfamiliar objects that were not readily named and that had no strongly associated context. Both older and younger adults demonstrated a typicality effect, showing significantly lower 2-alternative-forced-choice recognition of context-typical than context-atypical objects; for these objects, the recognition of older adults either significantly exceeded, or numerically surpassed, that of younger adults. Testing-awareness elevated recognition but did not interact with age or with object type. Older adults showed significantly higher recognition for context-atypical objects than for unfamiliar objects that had no prior strongly associated context. The observation of a typicality effect in both age groups is consistent with preserved semantic schemata processing in aging. The incidental recognition advantage of older over younger adults for the context-typical and context-atypical objects may reflect aging-related differences in goal-related processing, with older adults under comparatively more novel circumstances being more likely to direct their attention to the external environment, or age-related differences in top-down effortful distraction regulation, with older individuals' attention more readily captured by salient objects in the environment. Older adults' reduced recognition of unfamiliar objects compared to context-atypical objects may reflect possible age differences in contextually driven expectancy violations. The latter finding underscores the theoretical and methodological value of including a third type of objects-that are comparatively neutral with respect to their contextual associations-to help differentiate between contextual integration effects (for schema-consistent objects) and expectancy violations (for schema-inconsistent objects).

Aspects of mineral composition of male and female Heterobranchus bidorsalis adults exposed to different concentrations of Bonny-light crude oil

Studies were carried out to assess some macro and trace elements of mineral composition of the male and female Heterobranchus bidorsalis adults exposed to graded concentrations (1.00-8.00m/L-1) of Bonny-light crude oil (BLCO). The experiment was monitored for 4 days (toxicity) and 42days (recovery) periods. Significant decreases (P < 0.05)in the sodium (Na), potassium (K), magnesium (Mg), calcium (Ca), phosphorus (P), zinc (Zn), iron (Fe), vanadium (Va), lead (Pb) and manganese (Mn) contents of the male H. bidorsalis corresponded with the increasing concentrations of BLCO. In contrast, the female fishes recorded significant increases (P < 0.05) in the values of the above elements in their tissues as the concentrations of BLCO increased. Furthermore, the values of Na, K, Mg, Ca, P, Zn, Fe, Va, Pb and Mn recorded in the male fishes where generally lower than those of their female counterparts and the control fish. Increased values of these elements were also recorded during the recovery periods (days 14, 28 and 42) of this study in the magnitudes of 15% at day 14, 20% at day 28 and 20% at day 42. This implied that the removal of crude oil stress during this period improved the quantity of these minerals deposited in the fish tissues. The highest percent proportion of Zn and the lowest proportion of Pb recorded in both male and female H. bidorsalis adults agreed with the report of other workers for other fish species. KEYWORDS: Heterobranchus bidorsalis, Mineral composition, Bonny-light crude oil, Toxicity, Recovery.

Streptococcus pyogenes Pneumonia in Adults: Clinical Presentation and Molecular Characterization of Isolates 2006-2015

Introduction In the preantibiotic era Streptococcus pyogenes was a common cause of severe pneumonia but currently, except for postinfluenza complications, it is not considered a common cause of community-acquired pneumonia in adults. Aim and Material and Methods This study aimed to identify current clinical episodes of S. pyogenes pneumonia, its relationship with influenza virus circulation and the genotypes of the involved isolates during a decade in a Southern European region (Gipuzkoa, northern Spain). Molecular analysis of isolates included emm, multilocus-sequence typing, and superantigen profile determination. Results Forty episodes were detected (annual incidence 1.1 x 100,000 inhabitants, range 0.29-2.29). Thirty-seven episodes were community-acquired, 21 involved an invasive infection and 10 developed STSS. The associated mortality rate was 20%, with half of the patients dying within 24 hours after admission. Influenza coinfection was confirmed in four patients and suspected in another. The 52.5% of episodes occurred outside the influenza seasonal epidemic. The 67.5% of affected persons were elderly individuals and adults with severe comorbidities, although 13 patients had no comorbidities, 2 of them had a fatal outcome. Eleven clones were identified, the most prevalent being emm1/ST28 (43.6%) causing the most severe cases. Conclusions S. pyogenes pneumonia had a continuous presence frequently unrelated to influenza infection, being rapidly fatal even in previously healthy individuals.

Pressure support versus T-tube for weaning from mechanical ventilation in adults

BackgroundMechanical ventilation is important in caring for patients with critical illness. Clinical complications, increased mortality, and high costs of health care are associated with prolonged ventilatory support or premature discontinuation of mechanical ventilation. Weaning refers to the process of gradually or abruptly withdrawing mechanical ventilation. the weaning process begins after partial or complete resolution of the underlying pathophysiology precipitating respiratory failure and ends with weaning success (successful extubation in intubated patients or permanent withdrawal of ventilatory support in tracheostomized patients).ObjectivesTo evaluate the effectiveness and safety of two strategies, a T-tube and pressure support ventilation, for weaning adult patients with respiratory failure that required invasive mechanical ventilation for at least 24 hours, measuring weaning success and other clinically important outcomes.Search methodsWe searched the following electronic databases: Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2012, Issue 6); MEDLINE (via PubMed) (1966 to June 2012); EMBASE (January 1980 to June 2012); LILACS (1986 to June 2012); CINAHL (1982 to June 2012); SciELO (from 1997 to August 2012); thesis repository of CAPES (Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior) (http://capesdw.capes.gov.br/capesdw/) (August 2012); and Current Controlled Trials (August 2012).We reran the search in December 2013. We will deal with any studies of interest when we update the review.Selection criteriaWe included randomized controlled trials (RCTs) that compared a T-tube with pressure support (PS) for the conduct of spontaneous breathing trials and as methods of gradual weaning of adult patients with respiratory failure of various aetiologies who received invasive mechanical ventilation for at least 24 hours.Data collection and analysisTwo authors extracted data and assessed the methodological quality of the included studies. Meta-analyses using the random-effects model were conducted for nine outcomes. Relative risk (RR) and mean difference (MD) or standardized mean difference (SMD) were used to estimate the treatment effect, with 95% confidence intervals (CI).Main resultsWe included nine RCTs with 1208 patients; 622 patients were randomized to a PS spontaneous breathing trial (SBT) and 586 to a T-tube SBT. the studies were classified into three categories of weaning: simple, difficult, and prolonged. Four studies placed patients in two categories of weaning. Pressure support ventilation (PSV) and a T-tube were used directly as SBTs in four studies (844 patients, 69.9% of the sample). in 186 patients (15.4%) both interventions were used along with gradual weaning from mechanical ventilation; the PS was gradually decreased, twice a day, until it was minimal and periods with a T-tube were gradually increased to two and eight hours for patients with difficult and prolonged weaning. in two studies (14.7% of patients) the PS was lowered to 2 to 4 cm H2O and 3 to 5 cm H2O based on ventilatory parameters until the minimal PS levels were reached. PS was then compared to the trial with the T-tube (TT).We identified 33 different reported outcomes in the included studies; we took 14 of them into consideration and performed meta-analyses on nine. With regard to the sequence of allocation generation, allocation concealment, selective reporting and attrition bias, no study presented a high risk of bias. We found no clear evidence of a difference between PS and TT for weaning success (RR 1.07, 95% CI 0.97 to 1.17, 9 studies, low quality of evidence), intensive care unit (ICU) mortality (RR 0.81, 95% CI 0.53 to 1.23, 5 studies, low quality of evidence), reintubation (RR 0.92, 95% CI 0.66 to 1.26, 7 studies, low quality evidence), ICU and long-term weaning unit (LWU) length of stay (MD -7.08 days, 95% CI -16.26 to 2.1, 2 studies, low quality of evidence) and pneumonia (RR 0.67, 95% CI 0.08 to 5.85, 2 studies, low quality of evidence). PS was significantly superior to the TT for successful SBTs (RR 1.09, 95% CI 1.02 to 1.17, 4 studies, moderate quality of evidence). Four studies reported on weaning duration, however we were unable to combined the study data because of differences in how the studies presented their data. One study was at high risk of other bias and four studies were at high risk for detection bias. Three studies reported that the weaning duration was shorter with PS, and in one study the duration was shorter in patients with a TT.Authors' conclusionsTo date, we have found evidence of generally low quality from studies comparing pressure support ventilation (PSV) and with a T-tube. the effects on weaning success, ICU mortality, reintubation, ICU and LWU length of stay, and pneumonia were imprecise. However, PSV was more effective than a T-tube for successful spontaneous breathing trials (SBTs) among patients with simple weaning. Based on the findings of single trials, three studies presented a shorter weaning duration in the group undergoing PS SBT, however a fourth study found a shorter weaning duration with a T-tube.

Water-based exercise for adults with asthma

BackgroundAsthma is a common condition characterised by airway inflammation and airway narrowing, which can result in intermittent symptoms of wheezing, coughing and chest tightness, possibly limiting activities of daily life. Water-based exercise is believed to offer benefits for people with asthma through pollen-free air, humidity and effects of exercise on physical function.ObjectivesTo evaluate the effectiveness and safety of water-based exercise for adults with asthma.Search methodsWe searched the Cochrane Airways Group Specialised Register of Trials (CAGR), the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), the Allied and Complementary Medicine Database (AMED), PsycINFO, the Latin American and Caribbean Health Science Information Database (LILACS), the Physiotherapy Evidence Database (PEDro), the System for Information on Grey Literature in Europe (SIGLE) and Google Scholar on 13 May 2014. We handsearched ongoing clinical trial registers and meeting abstracts of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the British Thoracic Society (BTS).Selection criteriaWe included all randomised controlled trials (RCTs) of adults with asthma comparing a water-based exercise group versus one or more of the following groups: usual care, land-based exercise, non-exercise.Data collection and analysisTwo review authors (AJG, VS) independently extracted data fromthe primary studies using a standard form developed for this purpose, which includes methods, participants, interventions and outcomes. We contacted trial authors to request additional data. Data were input by one review author and were double-checked by a second review author.Main resultsIn this systematic review, we provide a narrative synthesis of available evidence from three small studies including 136 adult participants. the studies were at high risk of bias. No meta-analysis was possible because of methodological and interventional heterogeneity between included studies. the primary outcomes of quality of life and exacerbations leading to use of steroids were not reported by these studies. for exacerbations leading to health centre/hospital visits, uncertainty was wide because a very small number of events was reported (in a single study). Secondary outcomes symptoms, lung function, changes in medication and adverse effects, where available, described for each included study. the overall quality of the studies was very low, and no clear differences were noted between water-based exercise and comparator treatments. Therefore, we remain very uncertain about the effects of water-based exercise for adults with asthma.Authors' conclusionsThe small number of participants in the three included studies, the clinical and methodological heterogeneity observed and the high risk of bias assessed mean that we are unable to assess the place of water-based exercise in asthma. Randomised controlled trials are needed to assess the efficacy and safety of water-based exercise for adults with asthma. for future research, we suggest greater methodological rigour (participant selection, blinding of outcome assessors, reporting of all outcomes analysed and registering of the study protocol).

Interactive support effects on career agency and occupational engagement among young adults.

The PIC model by Gati and Asher describes three career decision making stages: pre-screening, in-depth exploration, and choice of career options. We consider the role that three different forms of support (general career support by parents, emotional/instrumental support, and informational support) may play for young adults in each of these three decision-making stages. The authors further propose that different forms of support may predict career agency and occupational engagement, which are important career decision precedents. In addition, we consider the role of personality traits and perceptions (decision-making window) on these two outcomes. Using an online survey sample (N = 281), we found that general career support was important for career agency and occupational engagement. However, it was the combination of higher general career support with either emotional/instrumental support or informational support that was found to lead to both greater career agency and higher occupational engagement. Personality also played a role: Greater proactivity also led to greater occupational engagement, even when there was little urgency for participants to make decisions (window of decision-making was wide open and not restricted). In practical terms, the findings suggest that the learning required in each of the three PIC processes (pre-screening, in-depth exploration, choice of career options may benefit when the learner has access to the three support measures.

What children learn from adults’ utterances: an ephemeral lexical boost and persistent syntactic priming in adult–child dialogue

We show that children’s syntactic production is immediately affected by individual experiences of structures and verb–structure pairings within a dialogue, but that these effects have different timecourses. In a picture-matching game, three- to four-year-olds were more likely to describe a transitive action using a passive immediately after hearing the experimenter produce a passive than an active (abstract priming), and this tendency was stronger when the verb was repeated (lexical boost). The lexical boost disappeared after two intervening utterances, but the abstract priming effect persisted. This pattern did not differ significantly from control adults. Children also showed a cumulative priming effect. Our results suggest that whereas the same mechanism may underlie children’s immediate syntactic priming and long-term syntactic learning, different mechanisms underlie the lexical boost versus long-term learning of verb–structure links. They also suggest broad continuity of syntactic processing in production between this age group and adults.