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The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.
Associations between smell identification deficits (SID) and impairments in basic cognitive domains have been shown in patients with neuropsychiatric disorders.
We analyzed social and basic cognitive deficits and SID.
To assess differences in affective decision making tasks in patients with schizophrenia-spectrum disorders, their 1st degree relatives and healthy controls. Methods: We examined 51 patients with schizophrenia-spectrum disorders (49% female, age 33.1 years, SD 11), 21 first-degree relatives (61.9% female, age 49.5 years, SD 17.6, one affected, others non-affected) and 51 matched healthy controls (49% female, age 33 years, SD 12.1). Psychopathology was evaluated using the Positive and Negative Syndrome Scale (PANSS). Subjects were administered the University of Pennsylvania Smell Identification Test (UPSIT), the Facially Expressed Emotion Labelling (FEEL) test, the spatial span subtest of the Wechsler Memory Scale-Revised (WMS-R) and the Mehrfachwahl-Wortschatz Test (MWT-B).
Patients, controls and 1st degree relatives differed in age (p = 0.000), WMS-R (p = 0.000) and FEEL scores (p = 0.007). In healthy controls, patients and 1st degree relatives FEEL correlated with age (p = 0.005, p = 0.003, p = 0.004, respectively). In patients FEEL also correlated with MWT-B (p = 0.000), UPSIT (p = 0.000) and PANSS negative scores (p = 0.016); furthermore, UPSIT correlated with MWT-B (p = 0.001). In 1st degree relatives age correlated with WMS-R (p = 0.04) and FEEL (p = 0.004), both of which inter-correlated (p = 0.006).
We found that SID, basic and social cognition, i.e. affective decision-making processes, inter-correlate in patients with schizophrenia-spectrum disorders and are partly under the influence of negative symptoms. Some of these relationships can also be seen in 1st degree relatives of patients.
The development and maintenance of an alcohol addiction is a complex interaction between genetic and environmental factors. Genetic effects seem to contribute substantially to the risk of developing an addiction, but also to its course and patients’ responses to different treatments. Recent studies identified associations between polymorphisms in the genes of glutamate and μ-opioid receptors and addiction risk. Those receptors are of special interest, because they are targets of therapeutic agents, such as acamprosate and topiramate.
Objectives and aims
Several studies were conducted, in order to further determine the effects of genetic polymorphisms in glutamate and opioid receptor genes on addictive behavior, neural response to alcohol cues and relapse risk.
Genetic effects were investigated in samples of alcohol-dependent patients using functional imaging techniques, neuropsychological tests and follow-up investigation after standard clinical treatment. Data on clinical parameters, neuronal response to alcohol cues, functional neuronal connectivity and relapse risk were collected and analyzed.
Results demonstrate effects of genetic polymorphisms in glutamate and opioid receptors on neuronal response to alcohol cues in frontal and mesolimbic brain areas, subjective craving and time to first relapse. Current findings will be discussed in the light of existing evidence on the contribution of genetic effects to treatment outcome and patient stratification.
The investigation of genetic risk factors and mechanisms by which they affect addiction related phenotypes seems to be a promising tool to identify molecular treatment targets and predictors for successful treatment strategies.
Disclosure of interest
The authors have not supplied their declaration of competing interest.
Alcohol relapse is often occurring to regulate negative affect during withdrawal. On the neurobiological level, alcoholism is associated with gray matter (GM) abnormalities in regions that regulate emotional experience such as the orbitofrontal cortex (OFC). However, no study to our knowledge has investigated the neurobiological unpinning of affect in alcoholism at early withdrawal and the associations of OFC volume with long-term relapse risk.
One hundred and eighty-two participants were included, 95 recently detoxified alcohol dependent patients (ADP) and 87 healthy controls (HC). We measured affective states using the positive and negative affect schedule (PANAS). We collected T1-weighted brain structural images and performed Voxel-based morphometry (VBM).
Findings revealed GM volume decrease in alcoholics in the prefrontal cortex (including medial OFC), anterior cingulate gyrus, and insula. GM volume in the medial OFC was positively associated with NA in the ADP group. Cox regression analysis predicted that risk to heavy relapse at 6 months increases with decreased GM volume in the medial OFC.
Negative affect during alcohol withdrawal was positively associated with OFC volume. What is more, increased GM volume in the OFC also moderated risk to heavy relapse at 6 months. Reduced GM in the OFC poses as risk to recovery from alcohol dependence and provides valuable insights into transient negative affect states during withdrawal that can trigger relapse. Implications exist for therapeutic interventions signifying the OFC as a neurobiological marker to relapse and could explain the inability of ADP to regulate internal negative affective states.
While DSM-5 classified pathological gambling as an addictive disorder, there is debate as to whether ICD-11 should follow suit. The debate hinges on scientific evidence such as neurobiological findings, family history of psychiatric disorders, psychiatric comorbidity, and personality variables.
In the “Baden-Württemberg Study of Pathological Gambling”, we compared a group of 515 male pathological gamblers receiving treatment with 269 matched healthy controls. We studied differences in sociodemographic characteristics, gambling-related variables, psychiatric comorbidity (lifetime), family history of psychiatric conditions, as well as personality traits such as impulsivity (Barratt Impulsiveness Scale), sensation seeking (Zuckerman's Sensation Seeking Scale) and the NEO-FFI big five. Personality traits were validated in an age- and ethnicity-matched subsample of “pure” gamblers without any psychiatric comorbidity (including nicotine dependence). Data were analyzed using two-sample t-tests, Chi2 analyses, Fisher's exact test and Pearson correlation analysis, as appropriate. Bonferroni correction was applied to correct for multiple comparisons.
Only 1% of the gamblers had been diagnosed with an impulse control disorder other than gambling (ICD-10). Notably, 88% of the gamblers in our sample had a comorbid diagnosis of substance dependence. The highest axis I comorbidity rate was for nicotine dependence (80%), followed by alcohol dependence (28%). Early age of first gambling experience was correlated with gambling severity. Compared to first-degree relatives of controls, first-degree relatives of pathological gamblers were more likely to suffer from alcohol dependence (27.0% vs. 7.4%), pathological gambling (8.3% vs. 0.7%) and suicide attempts (2.7% vs. 0.4%). Significant group differences were observed for the NEO-FFI factors neuroticism, agreeableness and conscientiousness. Gamblers were also more impulsive than controls, but did not differ from controls in terms of sensation seeking.
Our findings support classifying pathological gambling as a behavioural addiction in the ICD-11. This decision will have a significant impact on the approaches available for prevention (e.g. age limits) and treatment.
F. Spahn, University of Potsdam Potsdam, GERMANY,
H. Hoffmann, University of Potsdam Potsdam, GERMANY,
H. Rein, University of Toronto Toronto, Ontario, CANADA,
M. Seiss, University of Potsdam Potsdam, GERMANY,
M. Sremčević, University of Colorado Boulder, Colorado, USA,
M.S. Tiscareno, SETI Institute Mountain View, California, USA
When, in 1610, Galileo Galilei directed his telescope at Saturn, he discovered some puzzling addenda on either side of that planet, changing their appearance over the course of a few years – and even more disturbing, at certain instants they seemed to disappear and then return. These appendages remained a scientific riddle for about half a century until Christian Huygens came up with a seemingly correct model – he proposed that a solid ring is girdling Saturn. In 1675, G. D. Cassini's detection of a division in Saturn's rings – the Cassini Division separating the outer A and inner B rings – questioned Huygens’ hypothesis of a solid ring.
Almost 200 years later, in his famous work, Maxwell (1859) proved that a solid ring cannot be a stable configuration, suggesting instead that a myriad of individual tiny satellites form the rings of Saturn. This theoretical prediction was later confirmed experimentally by J. E. Keeler, who measured Doppler frequency shifts on either side of Saturn's rings (Keeler, 1889, 1895), showing that individual ring particles encircle Saturn at Kepler speeds.
Since those studies in the nineteenth century, the mesoscopic particulate nature of Saturn's rings has been widely accepted. Since the prediction of a flat monolayer ring by Jeffreys (1947), mainly suggested by the frequent inelastic collisions among the ring particles, only a little has been said about the properties of ring particles themselves – their size distribution, composition, etc., and their evolution as a granular ensemble.
Hénon (1981), motivated by the Pioneer and Voyager space missions to the outer solar system in the late 1970s and early 1980s, assumed a broad size distribution of the ring particles in order to explain spacecraft observations of the dense rings of Saturn. Properties like the apparent thickness of the rings or the distribution of the widths of dilute or empty gaps have been addressed by an extended power-law to characterize the size distribution of the ring particles. The idea behind this approach is that, depending on its size, a ring particle (especially sub-kilometer or kilometer-sized boulders, hereafter called moonlets) should gravitationally carve density features in the surrounding ring matter.
People with severe mental illness (SMI) have a high risk of living socially excluded from the mainstream society. Policy initiatives and health systems aim to improve the social situation of people who suffer from mental health disabilities. The aim of this study was to explore the extent of social exclusion (employment and income, social network and social activities, health problems) of people with SMI in Switzerland.
Data from the Swiss Health Survey 2012 were used to compare the social exclusion magnitude of people with SMI with those suffering from severe physical illness, common mental illness and the general population.
With the exception of Instrumental Activities of Daily Living, we found a gradient of social exclusion that showed people with SMI to be more excluded than the comparison groups. Loneliness and poverty were widespread among people with SMI. Logistic regression analyses on each individual exclusion indicator revealed that people with SMI and people with severe physical illness were similarly excluded on many indicators, whereas people with common mental illness and the general population were much more socially included.
In contrast to political and health system goals, many people with SMI suffer from social exclusion. Social policy and clinical support should increase the efforts to counter exclusionary trends, especially in terms of loneliness and poverty.
Strategies for the involvement of primary care in the management of patients with presumed or diagnosed dementia are heterogeneous across Europe. We wanted to explore attitudes of primary care physicians (PCPs) when managing dementia: (i) the most popular cognitive tests, (ii) who had the right to initiate or continue cholinesterase inhibitor or memantine treatment, and (iii) the relationship between the permissiveness of these rules/guidelines and PCP's approach in the dementia investigations and assessment.
Key informant survey. Setting: Primary care practices across 25 European countries. Subjects: Four hundred forty-five PCPs responded to a self-administered questionnaire. Two-step cluster analysis was performed using characteristics of the informants and the responses to the survey. Main outcome measures: Two by two contingency tables with odds ratios and 95% confidence intervals were used to assess the association between categorical variables. A multinomial logistic regression model was used to assess the association of multiple variables (age class, gender, and perceived prescription rules) with the PCPs’ attitude of “trying to establish a diagnosis of dementia on their own.”
Discrepancies between rules/guidelines and attitudes to dementia management was found in many countries. There was a strong association between the authorization to prescribe dementia drugs and pursuing dementia diagnostic work-up (odds ratio, 3.45; 95% CI 2.28–5.23).
Differing regulations about who does what in dementia management seemed to affect PCP's engagement in dementia investigations and assessment. PCPs who were allowed to prescribe dementia drugs also claimed higher engagement in dementia work-up than PCPs who were not allowed to prescribe.
Ernietta plateauensis Pflug, 1966 is the type species of the Erniettomorpha, an extinct clade of Ediacaran life. It was likely a gregarious, partially infaunal organism. Despite its ecological and taxonomic significance, there has not been an in-depth systematic description in the literature since the original description fell out of use. A newly discovered field site on Farm Aar in southern Namibia has yielded dozens of specimens buried in original life position. Mudstone and sandstone features associated with the fossils indicate that organisms were buried while still exposed to the water column rather than deposited in a flow event. Ernietta plateauensis was a sac-shaped erniettomorph with a body wall constructed from a double layer of tubes. It possessed an equatorial seam lying perpendicular to the tubes. The body is asymmetrical on either side of this seam. The tubes change direction along the body length and appear to be constricted together in the dorsal part of the organism.
The superconducting synchrotron SIS100 of the FAIR accelerator project will provide heavy ion beams of highest intensities. SIS100 is the first synchrotron with a special design, optimized for the control of ionization beam loss. Ionization beam loss is the most pronounced loss mechanism at operation with high-intensity, intermediate charge state heavy ions. The new synchrotron layout comprises an ion catcher system, which in combination with a charge separator lattice shall suppress dynamic vacuum effects.
A prototype cryogenic ion catcher, including a dedicated cryostat has been designed, manufactured, and tested under realistic conditions with beams from the heavy-ion synchrotron SIS18 at GSI. The gas desorption induced by the impact of heavy ions on this cryocatcher has been measured. For the very first time, a rise of desorption yield with increasing beam energy has been observed. However, measurements at room temperature have confirmed the known decrease of the pressure rise in the investigated energy regime. A transition temperature of 18 K, underneath hydrogen is adsorbed, could be verified several times. The results are significant and used to predict the ionization beam loss at operation of SIS100 at full-beam intensity.
Introduction: Point of care ultrasound has become an established tool in the initial management of patients with undifferentiated hypotension. Current established protocols (RUSH, ACES, etc) were developed by expert user opinion, rather than objective, prospective data. We wished to use reported disease incidence to develop an informed approach to PoCUS in hypotension using a “4 F’s” approach: Fluid; Form; Function; Filling. Methods: We summarized the incidence of PoCUS findings from an international multicentre RCT, and using a modified Delphi approach incorporating this data we obtained the input of 24 international experts associated with five professional organizations led by the International Federation of Emergency Medicine. The modified Delphi tool was developed to reach an international consensus on how to integrate PoCUS for hypotensive emergency department patients. Results: Rates of abnormal PoCUS findings from 151 patients with undifferentiated hypotension included left ventricular dynamic changes (43%), IVC abnormalities (27%), pericardial effusion (16%), and pleural fluid (8%). Abdominal pathology was rare (fluid 5%, AAA 2%). After two rounds of the survey, using majority consensus, agreement was reached on a SHoC-hypotension protocol comprising: A. Core: 1. Cardiac views (Sub-xiphoid and parasternal windows for pericardial fluid, cardiac form and ventricular function); 2. Lung views for pleural fluid and B-lines for filling status; and 3. IVC views for filling status; B. Supplementary: Additional cardiac views; and C. Additional views (when indicated) including peritoneal fluid, aorta, pelvic for IUP, and proximal leg veins for DVT. Conclusion: An international consensus process based on prospectively collected disease incidence has led to a proposed SHoC-hypotension PoCUS protocol comprising a stepwise clinical-indication based approach of Core, Supplementary and Additional PoCUS views.