Highly efficient ketone body treatment in multiple acyl-CoA dehydrogenase deficiency-related leukodystrophy.

BACKGROUND Multiple acyl-CoA dehydrogenase deficiency- (MADD-), also called glutaric aciduria type 2, associated leukodystrophy may be severe and progressive despite conventional treatment with protein- and fat-restricted diet, carnitine, riboflavin, and coenzyme Q10. Administration of ketone bodies was described as a promising adjunct, but has only been documented once. METHODS We describe a Portuguese boy of consanguineous parents who developed progressive muscle weakness at 2.5 y of age, followed by severe metabolic decompensation with hypoglycaemia and coma triggered by a viral infection. Magnetic resonance (MR) imaging showed diffuse leukodystrophy. MADD was diagnosed by biochemical and molecular analyses. Clinical deterioration continued despite conventional treatment. Enteral sodium D,L-3-hydroxybutyrate (NaHB) was progressively introduced and maintained at 600 mg/kg BW/d (≈3% caloric need). Follow up was 3 y and included regular clinical examinations, biochemical studies, and imaging. RESULTS During follow up, the initial GMFC-MLD (motor function classification system, 0 = normal, 6 = maximum impairment) level of 5-6 gradually improved to 1 after 5 mo. Social functioning and quality of life recovered remarkably. We found considerable improvement of MR imaging and spectroscopy during follow up, with a certain lag behind clinical recovery. There was some persistent residual developmental delay. CONCLUSION NaHB is a highly effective and safe treatment that needs further controlled studies.

Die beispiellose Hitze und Dürre von 1540 - ein Katastrophenszenario

Die Hitze- und Dürreperioden der Jahre 2003 und 201 O in Westeuropa und Russland wurden als sehr seltene klimatische Anomalien gewertet, deren Intensität außerhalb früherer Erfahrungen lag. Die Studie 'An underestimated record breaking event - why summer 1540 was likely warmer than 2003' zeigt hingegen, dass die Hitzewelle von 1540 wahrscheinlich noch wesentlich extremer war. Um dieses Resultat zu überprüfen, wurden in WETTER et al. (2014) die Niederschlagsverhältnisse und der bekannte Bodentrockenheit-Temperatur Rückkoppelungsme- chanismus (soll dessication-temperature feedback) untersucht. Basierend auf Ober 300 zeitgenössischen Quellen historischer Dokumentendaten aus ganz Europa konnte eine beispiellose, beinahe ununterbrochene Trockenphase von 11 Monaten für West und Mitteleuropa rekonstruiert werden. Die Schätzungen der saisonalen und jährlichen Niederschlagsmengen liegen signifikant unter den 100-jährlichen Minima der Instrumentellen Messperiode. Diese Resultate werden durch unabhängige historische Dokumentendaten unterstützt, welche von sehr niedrigen Wasserständen bedeutender Oberflächengewässer, europaweiten Feuersbrünsten und schweren sozioökonomischen Folgen berichten. Es zeigte sich weiterhin, dass die bis dato gängigen Klimamodelle noch nicht in der Lage sind, solch extreme Anomalien zu simulieren.

Die Skala zur Erfassung des Sportlehrerverhaltens SESV

Einleitung Sportliche Aktivität ist für die körperliche und geistige Entwicklung von Kindern und Jugendlichen von enormer Bedeutung (Kubesch, 2002). Im Rahmen des Schulsports übt u.a. das Sportlehrerverhalten (u.a. Feedback, soziale Unterstützung) einen Einfluss auf die Sporteinstellung und die sportliche Leistung der Heranwachsenden aus (Brand, 2006). Das Ziel der vorliegenden Arbeit ist es, die Entwicklung eines Messverfahrens zur Erfassung verschiedener relevanter Aspekte des Sportlehrerverhaltens zu beschreiben. Das Messverfahren stellt eine Adaptation des Coaching Behavior Questionnaire dar (CBQ; Williams et al., 2003). Methode Der CBQ ist ein Fragebogen, der in seiner ursprünglichen, englischsprachigen Form von Sportlern ausgefüllt wird und sich aus 15 Items zusammensetzt, mittels derer Sportler die wahrgenommene Unterstützung durch ihren Trainer und dessen Emotionalität auf vierstufigen Likert-Skalen (von 1 – stimme überhaupt nicht zu bis 4 – stimme voll und ganz zu) beschreiben können. Der CBQ wurde für die vorliegende Fragestellung an den Schulkontext angepasst, so dass Schüler das Verhalten ihres Sportlehrers hinsichtlich dessen Unterstützung und dessen Emotionalität beschreiben, weshalb diese Adaptation im Folgenden als Skala zur Erfassung des Sportlehrerverhaltens (SESV) bezeichnet wird. Zunächst wurden die ursprünglichen 15 Items von zwei englischsprachigen Muttersprachlern unabhängig voneinander ins Deutsche übersetzt. In einem nächsten Schritt füllten N = 176 Gymnasiasten (M/Alter = 14.12, SD/Alter = 2.25; 101 weiblich) einen Fragebogen aus, der neben dem Sportlehrerverhalten die Sporteinstellung (Brand, 2006), die Wettkampfängstlichkeit (Brand, Ehrlenspiel & Graf, 2009) sowie die Sportnote der Schüler erfasste. Ergebnisse und Diskussion Die interne Konsistenz der SESV lag im zufriedenstellenden Bereich (α = .85). Die Ergebnisse einer exploratorischen Faktorenanalyse unterstützen die angenommene Faktorenstruktur der Skala. Ein eher negatives Sportlehrerverhalten ging mit einer eher negativen Sporteinstellung, höherer Wettkampfängstlichkeit sowie einer schlechteren Sportnote einher. Die SESV ermöglicht eine ökonomische Erfassung des Sportlehrerverhaltens.

Prospective Memory, Retrospective Memory, Executive Functions, and Metacognition: How they are linked longitudinally in young elementary school children

Succeeding in everyday activities often requires executive functioning (EF), metacognitive abilities (MC) and memory skills such as prospective memory (PM) and retrospective memory (RM). These cognitive abilities seem to gradually develop in childhood, possibly influencing each other during development. From a theoretical point of view, it is likely that they are closely interrelated, especially in children. Their empirical relation, however, is less clear. A model that links these cognitive abilities can help to better understand the relation between PM and RM and other cognitive processes. In this project we studied the longitudinal development of PM, RM, EF, and MC in 7-8 year old elementary school children across half a year. 119 second graders (MT1 = 95 months, SDT1, = 4.8 months) completed the same PM, RM, EF and MC tasks twice with a time-lag of 7 months. The developmental progression was analysed using paired t-tests, the longitudinal relationships were analysed using confirmatory factor analysis and all fit indices are in accordance with Hu and Bentler (1998). In general, performance improved significantly (ps < .001) and effect sizes ranged from .45 to .62 (Cohen’s d). CFA revealed a good model fit, c2(227, 119) = 242.56, p = .23, TLI = .973, CFI = .979, RMSEA = .024. At T1, significant cross-sectional links were found between PM T1 and RM T1, between PM T1 and EF T1, and between EF T1 and MC T1. Moreover, significant longitudinal links were found between EFT1 and PMT2 and between EFT1 and MCT2; EF T1 and RM T2 were marginally linked. Results underline previous findings showing that PM, RM, EF, and MC develop significantly during childhood, even within this short time period. Results also indicate that these cognitive abilities are linked not only cross-sectionally, but longitudinally. Most relevant, however, is the predictive role of EF for both metacognition and memory.

Longitudinal relationships between prospective memory, executive functions, and metacognition in young elementary school children

Prospective Memory (PM), executive functions (EF) and metacognition (MC) are relevant cognitive abilities for everyday functioning. They all seem to develop gradually in childhood and appear to be theoretically closely related; however, their empirical links remain unclear, especially in children. As a recent study revealed significant cross-sectional links between PM and EF, and a weaker but close link between PM and MC in 2nd graders (Spiess, Meier, & Roebers, submitted), this study focused on their short-term relationships and on their development. 119 children (MT1 =95 months, SDT1, = 4.8 months) completed the same tasks (one PM, three EF, one MC task) twice with a time-lag of 7 months. T-tests showed significant improvements in all tasks, except in the updating task. Different structural equation models were contrasted (AMOS); the best fitting model revealed that PMT2 was similarly predicted by PMT1 (r = .33) and EFT1 (r = .34). Additionally, EFT1 predicted MCT2 (r = .44), chi2(118, 119) = 128.91, p = .23, TLI = .968, CFI = .978, RMSEA = .028. Results show that PM, EF, and MC develop during childhood and also demonstrate that they are linked not only cross-sectionally, but longitudinally. Findings are discussed in a broader developmental framework.

Time-series analysis of Campylobacter incidence in Switzerland

SUMMARY Campylobacteriosis has been the most common food-associated notifiable infectious disease in Switzerland since 1995. Contact with and ingestion of raw or undercooked broilers are considered the dominant risk factors for infection. In this study, we investigated the temporal relationship between the disease incidence in humans and the prevalence of Campylobacter in broilers in Switzerland from 2008 to 2012. We use a time-series approach to describe the pattern of the disease by incorporating seasonal effects and autocorrelation. The analysis shows that prevalence of Campylobacter in broilers, with a 2-week lag, has a significant impact on disease incidence in humans. Therefore Campylobacter cases in humans can be partly explained by contagion through broiler meat. We also found a strong autoregressive effect in human illness, and a significant increase of illness during Christmas and New Year's holidays. In a final analysis, we corrected for the sampling error of prevalence in broilers and the results gave similar conclusions.

Push and Pull Motivations for Quitting: A three-wave investigation of predictors and consequences of turnover

We report a new analysis of data from a multi-year study, some of which were previously published in the current journal. A longitudinal sample of 380 computer specialists was followed over two years, yielding three measures each of job satisfaction, organizational commitment, and turnover intentions, as well as actual turnover, and reasons for leaving, at Times 2 and 3. Career paths were more diverse than the classical distinction between stayers and leavers implies. Furthermore, although the largest single group of leavers cited “push” reasons, conforming to the classical withdrawal model, a sizable number were attracted to another job (“pull motivation”). In a three-wave structural equation model, job (dis)satisfaction predicted turnover, while organizational commitment exerted its influence only via its association with job satisfaction. As expected, however, attitudes predicted turnover only for participants with push motivation. Quitting, in turn, predicted an improvement in both satisfaction and commitment, indicating that it paid off for the individual. The necessity to study consequences of turnover and to distinguish between different subgroups of stayers and leavers is emphasized.

Reciprocal Relationships between Leader–Member Exchange (LMX) and Job Satisfaction: A Cross‐Lagged Analysis

While previous research has mainly emphasised the importance of leader–member exchange (LMX) to job satisfaction, there is a lack of research on reciprocal relationships between job satisfaction and LMX. In this study, we not only suggest that good LMX increases job satisfaction, but that job satisfaction can also enhance high-quality supervisor–employee relationships. A full cross-lagged panel analysis was used to test reciprocal relationships between LMX and job satisfaction. Employees (N= 279) of a large information technology company filled out questionnaires at two times, with a time lag of 3 months. In line with our predictions, findings revealed a positive relationship between LMX and job satisfaction both at Time 1 and Time 2. Moreover, LMX at Time 1 predicted the increase of job satisfaction at Time 2, and job satisfaction at Time 1 predicted the increase of LMX at Time 2. The results demonstrate the need to consider reciprocal relationships between job satisfaction and LMX when explaining employees' workplace outcomes. Our findings are discussed in terms of positive psychology theory.

Influencing factors on the length of stay in lumbar spine surgery: analysis of the German spine registry

INTRODUCTION Spinal disc herniation, lumbar spinal stenosis and spondylolisthesis are known to be leading causes of lumbar back pain. The cost of low back pain management and related operations are continuously increasing in the healthcare sector. There are many studies regarding complications after spine surgery but little is known about the factors predicting the length of stay in hospital. The purpose of this study was to identify these factors in lumbar spine surgery in order to adapt the postoperative treatment. MATERIAL AND METHODS The current study was carried out as a post hoc analysis on the basis of the German spine registry. Patients who underwent lumbar spine surgery by posterior surgical access and with posterior fusion and/or rigid stabilization, whereby procedures with dynamic stabilization were excluded. Patient characteristics were tested for association with length of stay (LOS) using bivariate and multivariate analyses. RESULTS A total of 356 patients met the inclusion criteria. The average age of all patients was 64.6 years and the mean LOS was 11.9 ± 6.0 days with a range of 2-44 days. Independent factors that were influencing LOS were increased age at the time of surgery, higher body mass index, male gender, blood transfusion of 1-2 erythrocyte concentrates and the presence of surgical complications. CONCLUSION Identification of predictive factors for prolonged LOS may allow for estimation of patient hospitalization time and for optimization of postoperative care. In individual cases this may result of a reduction in the LOS.

Dada Zürich und die Tanzreform

In diesem Vortrag wurden die ästhetischen und ideologischen Gemeinsamkeiten und Verbindungen zwischen den Tanzreformern um Rudolf von Laban und der 1916 gegründeten Dada-Gruppe in Zürich um Hugo Ball beleuchtet. Diskutiert hat Mona De Weerdt die Gemeinsamkeiten der beiden Gruppierungen bezüglich Programmatik, Ideologie, künstlerischer Neuerungen, Intentionen sowie die gesellschaftspolitischen Implikationen ihrer Kunst. Ein Fokus lag auf den in den Medienmitteilungen und Programmheften und von Zeitzeugen als ‚kubistisch’ oder ‚abstrakt’ bezeichneten Dada-Tänzen, die an Spielstätten wie dem Cabaret Voltaire, in der Galerie Dada und dem Saal zur Kaufleuten gezeigt wurde. Insbesondere hervorgehoben wurde die problematische Quellenlage und Schwierigkeit der Rekonstruktion dieser performativen Dada-Veranstaltungen.

Ian Barbours Methodologie in Wissenschaft und Religion

Die Theology & Science Community, die vor einem halben Jahrhundert begründet worden ist, blüht und gedeiht. Von Anfang an lag dabei die Methodenfrage im Zentrum: wie kann man diese so verschiedenen Gebiete in Beziehung setzen? Dies soll im Folgenden unter Berücksichtigung von Fragen der Erkenntnistheorie, Fragen der Natur wissenschaftlicher und religiöser Sprache und Fragen der Theoriekonstruktion, Theoriewahl und Theorieverteidigung zu beantworten versucht werden.

Über Gewalt und Liebe: Transnationale Adoption in Österreich

Über Gewalt und Liebe: Transnationale Adoption in Österreich Julia Rehsmann Eine Adoption bedeutet meist den Bruch mit der leiblichen Familie – zu verstehen als ein „Akt der Gewalt“ – und die Inkorporation in ein neues Familiennetzwerk – ein „Akt der Liebe“. Kinder zu bekommen wird in unserer Gesellschaft als wichtiger Teil des Lebens gesehen und trotz Fortschritten in der Reproduktionsmedizin bleibt die Zahl international adoptierter Kinder global gesehen auf einem gleich hohen Niveau. Im Rahmen einer Adoption werden vorherrschende Konzepte von Verwandtschaft und Zugehörigkeit in Frage gestellt, da die Beziehung zwischen Eltern und Kind nicht auf biogenetischer, sondern auf sozialer, emotionaler und rechtlicher Verbundenheit basiert. Wenn Adoptionen zwischen verschiedenen Ländern zustande kommen und das Kind meist anders aussieht als die Adoptiveltern, tauchen unweigerlich Fragen zu Identität, Zu- gehörigkeit, race und Kultur auf. Antworten zu Fragen wie „Wer bin ich?“ und „Woher komme ich?“ sind im Falle von Adoption und insbesondere von transnationaler Adoption komplizierter und schwieriger zu finden als im Falle der klaren biogenetischen Eltern-Kind-Beziehung. Welche Bedeutung hat diese Migration junger Kinder vor allem aus armen Nationen in die wohlhabenderen Länder der Vereinigten Staaten und Europas? Welche Bedeutung haben die in den letzten Jahren vermehrt auftretenden Reisen von Adoptierten und ihren Familien in ihre jeweiligen Geburtsländer? Die Adoptionsfamilie widerspricht in gewisser Hinsicht dem Bild der Familie als Teil des privaten Lebens, da sie während des Adoptionsprozesses von Bereichen des öffentlichen Lebens (Institutionen, Agenturen, Gesetzen) beeinflusst und bestimmt wird. Transnationale Adoptionen stellen nicht nur Individuen, sondern auch staatliche Institutionen vor große Herausforderungen und verlangen nach neuen Strategien im Umgang mit neuen Familienstrukturen. Im Rahmen des Vortrags soll ein Überblick über das Phänomen in Österreich gegeben werden und in Bezug zu anderen sozialanthro-pologischen Arbeiten zum Thema gesetzt werden, wobei in der empirischen Forschung der Fokus auf Familien mit Kindern aus Äthiopien lag.

The evolution of scarab beetles tracks the sequential rise of angiosperms and mammals

Extant terrestrial biodiversity arguably is driven by the evolutionary success of angiosperm plants, but the evolutionary mechanisms and timescales of angiosperm-dependent radiations remain poorly understood. The Scarabaeoidea is a diverse lineage of predominantly plant- and dung-feeding beetles. Here, we present a phylogenetic analysis of Scarabaeoidea based on four DNA markers for a taxonomically comprehensive set of specimens and link it to recently described fossil evidence. The phylogeny strongly supports multiple origins of coprophagy, phytophagy and anthophagy. The ingroup-based fossil calibration of the tree widely confirmed a Jurassic origin of the Scarabaeoidea crown group. The crown groups of phytophagous lineages began to radiate first (Pleurostict scarabs: 108 Ma; Glaphyridae between 101 Ma), followed by the later diversification of coprophagous lineages (crown-group age Scarabaeinae: 76 Ma; Aphodiinae: 50 Ma). Pollen feeding arose even later, at maximally 62 Ma in the oldest anthophagous lineage. The clear time lag between the origins of herbivores and coprophages suggests an evolutionary path driven by the angiosperms that first favoured the herbivore fauna (mammals and insects) followed by the secondary radiation of the dung feeders. This finding makes it less likely that extant dung beetle lineages initially fed on dinosaur excrements, as often hypothesized.

Plate fixation of extra-articular fractures of the proximal phalanx: do new implants cause less problems?

BACKGROUND Limited range of finger motion is a frequent complication after plate fixation of phalangeal fractures. The purpose of this study was to evaluate the results of plate fixation of extra-articular fractures of the proximal phalanx using current low-profile mini-fragment-systems. METHODS From 2006 to 2012, 32 patients with 36 extra-articular fractures of the proximal phalanx of the triphalangeal fingers were treated with open reduction and plate fixation (ORPF) using 1.2 and 1.5 mm mini-fragment systems. Patients presenting with open fractures grade 2 and 3 or relevant laceration of adjacent structures were excluded from the study. We retrospectively evaluated the rate of mal-union or non-union after ORPF, the need for revision surgery, for plate removal, and for tenolysis. Data were analyzed for further complications with regard to infections or complex regional pain syndrome (CRPS). RESULTS No infections were noted. Five patients developed transient symptoms of CRPS. Six weeks postoperatively, total active finger motion (TAM) averaged 183°, and all 32 patients underwent formal hand therapy. At the latest follow-up or at the time of plate removal, respectively, the mean TAM improved to 213°. Extension lag of proximal interphalangeal joints was found in 67 % of all fractured fingers. Secondary surgery was necessary in 14 of 32 patients (2 corrective osteotomies, 12 plate removals including 7 procedures explicitly because of reduced mobility). CONCLUSIONS Despite of new implant designs significant problems persist. Adhesions of extensor tendons leading to limited range of finger motion are still the most frequent complications after ORPF of proximal phalangeal fractures, even in absence of significant soft-tissue damage. LEVEL OF EVIDENCE Therapeutic, Retrospective, Level IV.

Modern solar maximum forced late twentieth century Greenland cooling

The abrupt Northern Hemispheric warming at the end of the twentieth century has been attributed to an enhanced greenhouse effect. Yet Greenland and surrounding subpolar North Atlantic remained anomalously cold in 1970s to early 1990s. Here we reconstructed robust Greenland temperature records (North Greenland Ice Core Project and Greenland Ice Sheet Project 2) over the past 2100 years using argon and nitrogen isotopes in air trapped within ice cores and show that this cold anomaly was part of a recursive pattern of antiphase Greenland temperature responses to solar variability with a possible multidecadal lag. We hypothesize that high solar activity during the modern solar maximum (approximately 1950s–1980s) resulted in a cooling over Greenland and surrounding subpolar North Atlantic through the slowdown of Atlantic Meridional Overturning Circulation with atmospheric feedback processes.