191 research outputs found
Decisions concerning job-related spatial mobility and their impact on family careers in France and Germany
"Beruflich bedingte räumliche Mobilität stellt die Frage nach der Erwerbsarbeit von Männern und Frauen. Es geht dabei aber nicht nur um die Entscheidung für einen bestimmten Beruf oder für einen Karriereschritt. Mobilität kann auch eine Folge von Doppelerwerbstätigkeit eines Paares sein. Wir gehen der Frage nach, wie die Entscheidung bei bi-aktiven Paaren im Verhältnis zu mono-aktiven Paaren getroffen wird und inwieweit andere soziodemographische Faktoren, besonders die Anwesenheit von Kindern in der Familie, Mobilitätsentscheidungen beeinflussen. Wir vergleichen deutsche und französische Daten, die in einer europäischen in sechs Ländern durchgeführten Studie Job Mobilities and Family Lives (2007) erhoben wurden. Die Erwerbstätigkeit von Frauen und die Familienpolitik sind in beiden Ländern relativ unterschiedlich. Während Frankreich schon seit Jahren Mütter mit konkreten Maßnahmen unterstützt, die es ermöglichen einer Erwerbstätigkeit nachzugehen, ist die Familienpolitik in Deutschland erst kürzlich von dem mutterzentrierten Modell abgekommen, dass Frauen darin unterstützte zuhause zu bleiben und die Kinder zu erziehen. Die statistische Auswertung wird vervollständigt durch Ergebnisse aus zwei qualitativen Studien. Die eine wurde 2006 in Frankreich durchgeführt. Die Ergebnisse der zweiten 2001 in Deutschland durchgeführten Studie werden berichtet. Beide Studien interessieren sich für Mobilitätsformen, die mehrere Tage Abwesenheit von zuhause verlangen. Eine Typologie unterschiedlicher 'Familienkarrieren', die im Rahmen der französischen Studie ausgearbeitet worden ist, wird hier zur Analyse des deutschen und französischen Materials herangezogen. Die Paare unterscheiden sich in Bezug auf den Entscheidungsprozess bezüglich der Mobilität und dieser wiederum hat unterschiedliche Partnerschaftskonzepte als Grundlage." (Autorenreferat)"Job-related spatial mobility raises questions about women's and men's professional life. It does not always accompany a specific job or a promotion; it may also arise as the consequence of being in a dual-career couple. We will study how the decision is handled by bi-active couples, compared to couples who live according to the more classical 'male breadwinner model', and how other socio-demographic factors, especially the presence of children, influence the decision in favour of mobility. We will compare data on France and Germany drawn from the European Survey Job Mobilities and Family Lives (2007) realised in six European countries. Women's employment rates and family policies are not the same in the two countries. While France has for several years provided solutions to help women remain in the labour force while raising children, Germany only recently abandoned a mother-centred family policy which pushed women to stay at home while raising their children. Statistical data will be completed by results from two qualitative studies to see more precisely how job mobility is experienced concretely. One study was realised in France in 2006. Results from the other study in Germany in 2001 will be reported. Both place the accent on mobile people who spend several days a week away from home. A typology of 'family careers', developed in the French study and applied to the French and German data shows that couples differ with regard to the decision-making processes on mobility, which reveal different underlying partnership patterns." (author's abstract
Structural diversity in the borohydrido lanthanides series: first isolation and X Ray crystal structure of ionic [Sm(BH4)2(THF)5]+[Cp*'Sm(BH4)3]-
International audienceTwo new borohydrido complexes of samarium were prepared: [Sm(BH4)2(THF)5]+[Cp*'Sm(BH4)3]- (1) and Cp*'2Sm(BH4)(THF) (3) (Cp*' = C5Me4nPr). X Ray studies revealed that 1 displays an unprecedented ionic structure comprising a half samarocene moiety, whereas 3 is monomeric and bears a terminal BH4 ligand
Primary bone diffuse large B-cell lymphoma: a retrospective evaluation on 76 cases from French institutional and LYSA studies
The GRAVITY+ Project: Towards All-sky, Faint-Science, High-Contrast Near-Infrared Interferometry at the VLTI
The GRAVITY instrument has been revolutionary for near-infrared
interferometry by pushing sensitivity and precision to previously unknown
limits. With the upgrade of GRAVITY and the Very Large Telescope Interferometer
(VLTI) in GRAVITY+, these limits will be pushed even further, with vastly
improved sky coverage, as well as faint-science and high-contrast capabilities.
This upgrade includes the implementation of wide-field off-axis
fringe-tracking, new adaptive optics systems on all Unit Telescopes, and laser
guide stars in an upgraded facility. GRAVITY+ will open up the sky to the
measurement of black hole masses across cosmic time in hundreds of active
galactic nuclei, use the faint stars in the Galactic centre to probe General
Relativity, and enable the characterisation of dozens of young exoplanets to
study their formation, bearing the promise of another scientific revolution to
come at the VLTI.Comment: Published in the ESO Messenge
Bone Marrow Transplant
Mucopolysaccharidosis type I-H (MPS I-H) is a rare lysosomal storage disorder caused by α-L-Iduronidase deficiency. Early haematopoietic stem cell transplantation (HSCT) is the sole available therapeutic option to preserve neurocognitive functions. We report long-term follow-up (median 9 years, interquartile range 8-16.5) for 51 MPS I-H patients who underwent HSCT between 1986 and 2018 in France. 4 patients died from complications of HSCT and one from disease progression. Complete chimerism and normal α-L-Iduronidase activity were obtained in 84% and 71% of patients respectively. No difference of outcomes was observed between bone marrow and cord blood stem cell sources. All patients acquired independent walking and 91% and 78% acquired intelligible language or reading and writing. Intelligence Quotient evaluation (n = 23) showed that 69% had IQ ≥ 70 at last follow-up. 58% of patients had normal or remedial schooling and 62% of the 13 adults had good socio-professional insertion. Skeletal dysplasia as well as vision and hearing impairments progressed despite HSCT, with significant disability. These results provide a long-term assessment of HSCT efficacy in MPS I-H and could be useful in the evaluation of novel promising treatments such as gene therapy
A Solve-RD ClinVar-based reanalysis of 1522 index cases from ERN-ITHACA reveals common pitfalls and misinterpretations in exome sequencing
Purpose
Within the Solve-RD project (https://solve-rd.eu/), the European Reference Network for Intellectual disability, TeleHealth, Autism and Congenital Anomalies aimed to investigate whether a reanalysis of exomes from unsolved cases based on ClinVar annotations could establish additional diagnoses. We present the results of the “ClinVar low-hanging fruit” reanalysis, reasons for the failure of previous analyses, and lessons learned.
Methods
Data from the first 3576 exomes (1522 probands and 2054 relatives) collected from European Reference Network for Intellectual disability, TeleHealth, Autism and Congenital Anomalies was reanalyzed by the Solve-RD consortium by evaluating for the presence of single-nucleotide variant, and small insertions and deletions already reported as (likely) pathogenic in ClinVar. Variants were filtered according to frequency, genotype, and mode of inheritance and reinterpreted.
Results
We identified causal variants in 59 cases (3.9%), 50 of them also raised by other approaches and 9 leading to new diagnoses, highlighting interpretation challenges: variants in genes not known to be involved in human disease at the time of the first analysis, misleading genotypes, or variants undetected by local pipelines (variants in off-target regions, low quality filters, low allelic balance, or high frequency).
Conclusion
The “ClinVar low-hanging fruit” analysis represents an effective, fast, and easy approach to recover causal variants from exome sequencing data, herewith contributing to the reduction of the diagnostic deadlock
Une lecture sociologique de la gastronomie : processus de régulation, de pressions et de contrôles entre cuisiniers, critiques gastronomiques et clients
L'analyse de situations à l'épreuve des scénarios. L'exemple des actions qualité dans l'industrie
Féminisation et féminités dans les métiers à dominante technique d'une grande entreprise de transport
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