Hydraulic and thermal impact modelling at the scale of the geothermal heating doublet in the Paris Basin, France

13 pagesInternational audienceThe Paris Basin is extensively developed for the geothermal district heating (GDH) of approximately 150 000 dwellings. As of late 2010, thirty four GDH systems apply the doublet concept in the Paris suburban area and mine the heat of the Dogger reservoir, a limestone formation of Mid-Jurassic age at depths ranging from 1500 to 2000 m. As the brine is fully reinjected, cold water bodies progressively invade the reservoir around injector wells inducing both thermal and hydraulic interactions at the doublet scale. The premature production well cooling and the sustainable development of the resource highlight two critical parameters, the thermal breakthrough time (tB) and the extent of the cooled fluid bubble(S) respectively. First, a set of benchtest simulations was launched to compare the sensitivities of tB and S parameters to selected reservoir conceptual model typologies. These simulations were applied on a GDH doublet undergoing a suspected thermal breakthrough. Five reservoir modelling teams validated their “in house” simulations by (i) checking an analytical (Gringarten-Sauty, 1979) solution, and (ii) testing three candidate reservoir structures on the doublet considered remotely located (i.e. not interfering with nearby exploitations) for a first step. The outcome resulted in a rewarding insight into the variability of simulation outputs. An additional segment will enable the actors to compare their modelling expertise on the same doublet considered in interaction with the other GDH operations located in its environment. Second, BRGM carried out a survey towards various rehabilitation schemes (a new doublet or a triplet) and their contribution toward sustainability standards. From a hypothetical, twenty five year life, doublet simulation, an initial hydraulic/temperature field was derived. Then, several new well locations were simulated and isotherms, alongside production well cooling kinetics, compared accordingly. A two-stage rehabilitation scheme, i.e. triplet then a new doublet, seems to reconcile the resource longevity and the economic demand. Further work is required to compare the different designs with a method integrating both the impact of the geothermal exploitation on the resource and the lifetime of the exploitation in a single mathematical factor

Finger creases lend a hand in Kabuki syndrome.

International audienceKabuki syndrome (KS) is a rare syndrome associating malformations with intellectual deficiency and numerous visceral, orthopedic, endocrinological, immune and autoimmune complications. The early establishment of a diagnostic of KS leads to better care of the patients and therefore prevents complications such as perception deafness, severe complications of auto-immune diseases or obesity. However, the diagnosis of KS remains difficult because based on the appreciation of facial features combined with other highly variable features. We describe a novel sign, namely the attenuation and/or congenital absence of the IPD crease of the third and fourth fingers associated with limitation of flexion of the corresponding joints, which seems to be specific of KS and could help the clinician to diagnose KS

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

Historique de la coopération franco-chinoise

Le partenariat entre la Chine et la France sur le nucléaire se caractérise avant tout par sa flexibilité et son adaptation – comment pourrait-il en être autrement au vu des transformations spectaculaires de la Chine au cours de ces trente dernières années ? Il se distingue également par sa profondeur et sa richesse, touchant directement ou indirectement la vie de centaines de personnes, ingénieurs, chercheurs, dirigeants, des deux pays. Ce partenariat comprend plusieurs facettes, allant de la R&D à la sûreté, et intégrant une dimension industrielle et régalienne. Sa vitalité est une autre de ses caractéristiques. Cette année, la montée en puissance de nombreux projets consolidera ce partenariat unique

Le nucléaire Français en Chine : 30 ans de collaboration

Le Nucléaire Chinois traverse actuellement une période de transition, dans un contexte marqué par les évènements de Fukushima : ralentissement temporaire des constructions, passage progressif à des réacteurs répondant aux objectifs de sûreté de la troisième génération, sensibilité accrue du public sur la question nucléaire. Le plan de développement du nucléaire à long terme reste cependant ambitieux et inchangé, et 30 ans après le début de leur collaboration, la Chine et la France entretiennent des liens toujours très forts dans ce domaine, tant au niveau industriel que de la R&D, dans l'amont, l'aval, les réacteurs, sans oublier la sûreté

Le mélange sérum salé hypertonique-hydroxyéthylamidon administré après un choc hémorragique ne dégrade pas la fonction respiratoire sur un modèle porcin de contusion pulmonaire

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-Bib. Serv.Santé Armées (751055204) / SudocSudocFranceF

Small Modular Reactors en Finlande : l’avenir du chauffage urbain décarboné ?

Soucieuse de construire une société bas carbone, la Finlande entend réduire de 80 à 95 %, à l’horizon 2050, ses émissions de gaz à effet de serre par rapport au niveau de 1990. D’ici à 2029, le charbon sera interdit et les énergies fossiles verront leur poids dans le mix énergétique très largement réduit

Conscience et anesthésie

International audienc

Persistent cardiac rhabdomyoma in an adult with tuberous sclerosis

International audienc

Syndrome d'emphysème des sommets et fibrose pulmonaire des bases combinés

National audienceKey points A syndrome including upper-lobe emphysema and pulmonary fibrosis of the lower lungs was recently characterized. It is found most often in men who are smokers or ex-smokers of more than 40 pack-years; their mean age is 65 years. Exertional dyspnea is always present. There are basal crackles. The disease has no known cause; the only certain risk factor is smoking. Pulmonary function tests show respiratory volumes and flows that are often normal or subnormal, while carbon monoxide transfer is substantially reduced and exercise hypoxemia is present. Diagnosis is based on findings from millimeter-slices of computed tomography of the chest, which show either centrilobular emphysema or upper-zone bullous emphysema, associated in 90% of cases with very suggestive paraseptal emphysema and diffuse infiltrating fibrosing lung disease at the bases (subpleural reticular opacities, honeycomb images, traction bronchiectasis), with more frequent ground glass opacities than in idiopathic pulmonary fibrosis. Pulmonary hypertension is present in almost half of all patients and represents the principal negative prognostic factor for this condition, which has a median survival of 6 years.Points essentiels Un syndrome comportant un emphysème des sommets combiné à une fibrose pulmonaire des bases a été récemment individualisé. Il se rencontre majoritairement chez des sujets de sexe masculin, fumeurs ou anciens fumeurs à plus de 40 paquets-années, et âgés en moyenne de 65 ans. La dyspnée d'effort est constante. Il existe des râles crépitants des bases pulmonaires. La maladie n'a pas de cause connue ; le seul facteur de risque formel est le tabagisme. L'exploration fonctionnelle respiratoire montre des volumes et débits pulmonaires souvent normaux ou subnormaux, contrastant avec un abaissement majeur du transfert du monoxyde de carbone, et une hypoxémie d'exercice. Le diagnostic repose sur le scanner thoracique en coupes millimétriques, qui montre un emphysème centrolobulaire et/ou bulleux des sommets pulmonaires, associé dans 90 % des cas à un emphysème paraseptal très évocateur, et une pneumopathie infiltrante diffuse fibrosante prédominant dans les bases (opacités réticulaires sous-pleurales, images en rayons de miel, bronchectasies par traction), comportant des opacités en verre dépoli plus fréquentes qu'au cours de la fibrose pulmonaire idiopathique. Une hypertension pulmonaire est présente chez près de la moitié des malades, et représente le principal facteur pronostique péjoratif de cette affection dont la survie médiane est de 6 ans