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International audience; Contexte: Le déficit en alpha 1-antitrypsine (DAAT) est une maladie génétique autosomique récessive associée à l’état homozygote du variant Z du gène SERPINA1. Les manifestations cliniques sont un déficit sévère en alpha 1-antitrypsine, un emphysème pulmonaire et une fibrose hépatique.Méthodes: Des recommandations de prise en charge du DAAT ont été élaborées à l’initiative du centre coordonnateur de référence des maladies pulmonaires rares, sous l’égide de la Société de pneumologie de langue française, par des groupes de coordination, rédaction, et lecture, impliquant des pneumologues de divers modes d’exercice, biologistes, hépatologue, pharmacien hospitalier, conseiller en génétique, radiologues, médecins généralistes, cadre de santé, et associations de patients. La méthode d’élaboration des « Recommandations pour la pratique clinique » de la Haute autorité de santé a été suivie, incluant un vote en ligne selonune échelle Likert.Résultats: Après une analyse bibliographique, 20 recommandations ont été proposées par les groupes de travail, portant sur tous les aspects de la maladie : diagnostic biologique, bilan initial, conseils d’hygiène de vie, information, indications et modalités du traitement substitutif, dépistage.Conclusion: Ces recommandations fondées sur les preuves sont destinées à guider le diagnostic et la prise en charge pratique du DAAT.

Une étude analytique de la maturation de la baie de raisin en Champagne est envisagée sur 4 ans et 3 cépages. Les résultats permettent de proposer un modèle de prévision d'une date de vendange ainsi qu'une estimation de la qualité de la vendange en ce qui concerne les sucres. Investigations on grapevine maturation in the Champagne region: Method of forecasting the harvesting date An analytic investigation on the maturation of grape berries in the Champagne region was carried out on 3 cultivars over 4 years. The results enable us to offer a model for estimating harvesting dates and to foresee the vintage quality as far as sugar is concerned., VITIS - Journal of Grapevine Research, Vol. 22 No. 2 (1983): Vitis

This paper addresses the use of Big Data and machine learning based analytics to the real-time management of Internet scale Quality-of-Service Route Optimisation with the help of an overlay network. Based on the collection of large amounts of data sampled each $2$ minutes over a large number of source-destinations pairs, we show that intercontinental Internet Protocol (IP) paths are far from optimal with respect to Quality of Service (QoS) metrics such as end-to-end round-trip delay. We therefore develop a machine learning based scheme that exploits large scale data collected from communicating node pairs in a multi-hop overlay network that uses IP between the overlay nodes themselves, to select paths that provide substantially better QoS than IP. The approach inspired from Cognitive Packet Network protocol, uses Random Neural Networks with Reinforcement Learning based on the massive data that is collected, to select intermediate overlay hops resulting in significantly better QoS than IP itself. The routing scheme is illustrated on a $20$-node intercontinental overlay network that collects close to $2\times 10^6$ measurements per week, and makes scalable distributed routing decisions. Experimental results show that this approach improves QoS significantly and efficiently in a scalable manner.

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The temporal evolution of the main compounds present in the phloem sap feeding the cluster of Vitis vinifera Pinot noir has been determined from the beginning of flowering until fruit set, after improvement of the facilitated exudation technique. The retained composition for the dipping solution was: HEPES (10 mM, pH 7.5), EDTA (10 mM). The first ramification of the cluster, maintained in situ, was sectionned then immersed in the dipping solution in order to favour the phloem exudation. The major organic components of the phloem sap were carbohydrates, amino acids and organic acids (i.e. sucrose, glutamine and tartrate, respectively). For each metabolic group, the mean exuded quantities correspond to 300 nmol per cluster in 4 h. The sharp increase in both organic compounds and potassium released over the flowering time-course reflects the rise of the mass flow supplying the cluster and underline the increasing sink strength of this organ. Moreover, the increasing contents of glutamine and hexoses in the exudate suggest a regulation in the allocation of assimilates to the reproductive organs. Les exsudats de sève phloémique comme critère d'appreciation de la force de puits de la grappe chez Vitis vinifera cv. Pinot noir L'évolution temporelle des principaux constituants de la sève libérienne alimentant la grappe de Vitis vinifera Pinot noir a été étudiée au cours de la floraison, après adaptation d'une technique de prélèvement par exsudation facilitée. La composition de la solution d'exsudation retenue est la suivante: HEPES (10 mM, pH 7,5), EDTA (10 mM). Sur la grappe maintenue in situ, l'extrémité de la première ramification est sectionnée puis immergée dans le milieu précédemment défini pour permettre la récupération des assimilats. Les composés organiques prédominants dans la sève libérienne sont les glucides solubles, les acides aminés et les acides organiques (saccharose, glutamine et tartrate respectivement). Pour chacun de ces groupes métaboliques, les quantités moyennes exsudées sont voisines de 300 nmol par grappe en 4 heures. Les quantités croissantes de glucides, d'acides aminés et de potassium collectées entre Je début de l'anthèse et la nouaison reflètent l'augmentation de flux de masse parvenant à la grappe et soulignent l'évolution de la force d'appel de ce puits. De plus, la part croissante de la glutamine et des hexoses dans les exsudats suggère une régulation dans la distribution des assimilats aux organes reproducteurs., VITIS - Journal of Grapevine Research, Vol. 31 No. 3 (1992): Vitis

National audience; The authors describe 2 cooling chambers used to study frost sensitivity of vine buds. The first is a classical convective cooling system which can be run in the field (fig 1). The second is designed to provide buds with the radiative cooling which occurs during spring frosts. This chamber reproduces part of the system designed by Went (1976), Gill and Waister (1976) and Marcellos (1981). Figure 3 reproduces the schema of the chamber. Radiative cooling is provided by means of evaporating nitrogen at roof level in the upper part (3). Warm air is circulated in the middle part in order to maintain the polyethylene walls (4) free of water and transparent to thermal infrared radiation. This radiative cooling system makes dew or rime deposition possible, as in natural conditions. This is illustrated in figure 4 where bud temperature (----- and ---) is kept lower than both air temperature (-) and dew point (-·-) during cooling.; Les auteurs décrivent 2 chambres de refroidissement utilisées pour les études de gélivité des bourgeons in situ. La première est une chambre de refroidissement convectif élaborée à partir d’éléments de congélateurs. La deuxième cherche à reproduire le phénomène de refroidissement radiatif qui a lieu au cours des nuits de gelées de printemps afin de permettre les dépôts de rosée et de givre qui se produisent dans les conditions naturelles.

National audience; An experiment on sensitivity to spring frost in vine buds was undertaken in the champagne vineyard (Vitis vinifera L Chardonnay) during the springs of 1987-1989 by means of differential thermal analysis (first exotherm) performed in freezing chambers, providing either convective cooling (on vine directly in the field) or radiative cooling on twigs. The sensitivity of buds was analysed in terms of bud phase, surface wetness and ice nuclei bacteria (Pseudomonas syringae) concentration. Figure 1 shows the evolution with phenological phase of mean freezing point of dry buds for 3 yr independently of ice nuclei concentration. Figure 2 reports this freezing point for dry vine buds which contain < 103 bacteria, ie quasi-free of ice nuclei. Figure 3 reports for stage 9 of Eichhhorn and Lorenz the freezing point of dry buds versus ice nuclei concentration. The evolution of the mean freezing point at different stages is plotted on figure 4 versus mean ice nuclei bacterial concentration for both dry and wetted vine buds. From this figure, one can see that frost sensitivity of buds is increased not only by phenology but also by wetness of bud surface. On the contrary, the influence of ice nuclei bacteria is not so obvious. It seems that this bacterial influence could be different depending on the origin of the bacteria. In figure 5 at stage 7, the freezing point of contaminated buds (x) is higher than that of buds which contain the same amount of bacteria (Δ). The only minor effect of natural ice nuclei bacteria concentration on buds was observed on the percentage of surviving buds for a given temperature : the concentration of bacteria observed on killed buds (formula, see attached document) is a little higher in figure 6 than those in surviving buds (+).; Une étude expérimentale de la gélivité des bourgeons de vigne in situ a été entreprise sur le vignoble champenois (Vitis vinifera L Chardonnay) de 1987 à 1989 en utilisant l’analyse thermique différentielle dans des chambres de refroidissement. L’analyse de la gélivité a consisté à étudier l’influence sur la température de rupture de surfusion de facteurs tels que le stade phénologique, le mouillage des organes végétaux et la teneur en bactéries dites glaçogènes. Au vu des résultats il apparaît que stade phénologique et mouillage sont des variables déterminantes, tandis que l’action des bactéries est beaucoup plus ténue et difficile à interpréter. Les informations recueillies doivent permettre à terme d’élaborer un modèle probabiliste du risque de rupture de surfusion dans le bourgeon.

This document brings together the findings of a number of studies on the informal sector, and provides an analysis of the issues and possible policy options for the informal sector in Guinea. The book starts with a synthesis of the informal sector issue and the links with the modern sector in the Guinean context. It discusses the country's past, the different economic and financial reforms and the structural adjustment programme. Various studies of the informal sector are presented, as well as the organisations for the promotion of the informal sector and those dealing with vocational training. The sector's contribution to the country's employment and national product, the human resources involved and the skill acquisition are discussed. Management and organisational problems of small firms, obstacles to their growth and financial aspects, including taxation are analysed. The concluding chapter calling for a recognition of informal sector issues contains proposals for preliminary assistance to the sector and a general policy including sectoral approach, extension to rural non-farm activities, suitable financial and fiscal policy.