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Resume L’emergence de bacteries multiresistantes aboutissant a des impasses therapeutiques fait de la bonne gestion des antibiotiques une priorite internationale de sante publique. L’utilisation des antibiotiques dans un but de prevention (antibioprophylaxie) doit donc etre raisonnee et guidee par les preuves. Les mesures d’antibioprophylaxie doivent toujours s’accompagner de mesure de prevention des infections (vaccination quand cela est indique, respect strict des regles d’hygiene). Il existe deux grandes categories d’antibioprophylaxie : l’antibioprophylaxie chirurgicale et l’antibioprophylaxie medicale. La premiere a montre son efficacite pour la prevention des infections de sites operatoires dans des situations bien definies. Elle repose sur le principe d’obtention d’une concentration d’antibiotique suffisante au site d’incision pour eviter que les bacteries presentes penetrent dans le site operatoire et y proliferent. Le choix de l’antibiotherapie varie en fonction des sites operatoires et du type de chirurgie. L’antibioprophylaxie chirurgicale ne concerne pas la chirurgie de site infecte et doit etre limitee dans le temps avec, le plus souvent, une seule injection perioperatoire et toujours d’une duree maximale de 48 heures. L’antibiotherapie medicale peut etre indiquee dans certaines situations tres bien definies comme certaines situations d’immunodepression (asplenie, deficit de l’immunite cellulaire severe), la postexposition a des infections invasives (meningococcemie, coqueluche, infection invasive a streptocoque du groupe A, morsure) ou a des situations a risques (certains gestes invasifs chez des patients porteurs de cardiopathies a risques). L’indication de l’antibioprophylaxie urinaire est de plus en plus restreinte au vu de la balance benefice/risque et doit toujours etre discutee avec un specialiste.

Rates of perceived fatigue, anxiety, depression, sleepiness and mitochondrial disease severity were assessed prospectively in 2017-2018 using established validated questionnaires in 48 adult patients with genetically confirmed primary mitochondrial disease. Fatigue was found to be very common among patients with primary mitochondrial disease, with 34 to 48 (71-100%) patients reporting fatigue depending on the measure used, and the severity of fatigue correlating with the severity of disease. Moderate-to-severe depression (10/48; 20.8%) anxiety (28/48; 58.3%) and sleep problems (16/48; 33.3%) were frequent in our patients with fatigue and these conditions were even more prevalent in those with severe fatigue. In conclusion, perceived fatigue was common in patients with primary mitochondrial disease and appeared to correlate with disease severity. Depression, anxiety and sleep disorders were more common in the cohort than those with other chronic diseases but with rates similar to that seen in multiple sclerosis. The severity of perceived fatigue correlated with an increased risk of these comorbid conditions. The Fatigue Severity Scale may more selectively measure non-anxiety/sleep-related fatigue in primary mitochondrial disease and additional testing is planned.

Cerebellar ataxia is a hallmark of coenzyme Q10 (CoQ10) deficiency associated with COQ8A mutations. We present four patients, one with novel COQ8A pathogenic variants all with early, prominent handwriting impairment, dystonia and only mild ataxia. To better define the phenotypic spectrum and course of COQ8A disease, we review the clinical presentation and evolution in 47 reported cases. Individuals with COQ8A mutation display great clinical variability and unpredictable responses to CoQ10 supplementation. Onset is typically during infancy or childhood with ataxic features associated with developmental delay or regression. When disease onset is later in life, first symptoms can include: incoordination, epilepsy, tremor, and deterioration of writing. The natural history is characterized by a progression to a multisystem brain disease dominated by ataxia, with disease severity inversely correlated with age at onset. Six previously reported cases share with ours, a clinical phenotype characterized by slowly progressive or static writing difficulties, focal dystonia, and speech disorder, with only minimal ataxia. The combination of writing difficulty, dystonia and ataxia is a distinctive constellation that is reminiscent of a previously described clinical entity called Dystonia Ataxia Syndrome (DYTCA) and is an important clinical indicator of COQ8A mutations, even when ataxia is mild or absent.

Resume Objectifs Presenter les recommandations de pratique clinique (RCP) de la Societe francaise d’otorhinolaryngologie–chirurgie de la face et du cou concernant l’utilisation des AINS dans les infections ORL pediatriques. Methodes A partir d’une analyse critique de la litterature medicale jusqu’en novembre 2016, un groupe de travail multidisciplinaire constitue de 11 praticiens a redige des recommandations de pratique clinique classees en categories Grades of Recommendation, Assessment, Development and Evaluation (GRADE) A, B, C, ou accord professionnel selon un niveau de preuve scientifique decroissant. La premiere version du texte a ete remaniee par le groupe de travail a la suite des remarques des 22 membres du groupe de lecture avant de produire le texte definitif. Resultats Les principales recommandations issues de ce travail sont les suivantes : (a) Les AINS sont indiques a posologies antalgiques 20 a 30 mg/kg/j en association avec le paracetamol dans les infections ORL pediatriques non compliquees (otites moyennes aigues, angines, rhinopharyngites, sinusites maxillaires) si : (i) les douleurs sont d’intensite moyenne (score de l’echelle visuelle analogique ou EVA compris entre 3 et 5 ou score « Evaluation Enfant Douleur » ou EVENDOL compris entre 4 et 7) et insuffisamment calmees par le paracetamol prescrit en premiere intention (EVA restant ≥ 3 ou EVENDOL restant ≥ 4) ; (ii) les douleurs sont moderees a intenses (EVA entre 5 et 7 ou EVENDOL entre 7 et 10) ; (b) Lorsqu’ils sont associes, paracetamol et ibuprofene sont idealement pris simultanement toutes les 6 heures ; (b) il est recommande de : (i) ne pas prescrire d’AINS dans les infections ORL pediatriques severes ou compliquees ; (ii) suspendre le traitement par AINS en cas de tableau clinique infectieux inhabituel par sa duree ou par sa symptomatologie ; (iii) limiter dans tous les cas la duree de prescription des AINS a 72 h.

Successive implementation of seven-valent then 13-valent pneumococcal conjugate vaccines (PCVs) led to a marked decrease in pneumococcal disease burden, including pneumococcal meningitis. We assessed the long-term effect of implementation of PCVs on incidence of pneumococcal meningitis in children in France over a 16-year period.We did a quasi-experimental, population-based interrupted time-series analysis with a nationwide prospective survey over 16 years in France, recruiting children aged younger than 15 years from 227 paediatric wards from January, 2001, to December, 2016. The main outcome by the time-series model was the estimated incidence of pneumococcal meningitis per 100 000 children (of a population of 12·6 million children in 2017) before and after PCV7 and PCV13 implementation. The time-series model was based on segmented regression with autoregressive error.We enrolled 1778 children with pneumococcal meningitis. PCV13 implementation led to a significant reduction in monthly incidence of pneumococcal meningitis from 0·12 per 100 000 children before PCV13 to a nadir of 0·07 in December, 2014 (-38%, 95% CI -56·1 to -20·4; p0·0001). A sharp increase occurred during 2015 and 2016, (+2·3% per month, incidence of 0·13 per 100 000 children at the end of the study period, p=0·0002), mainly related to an increase of serotype 24F, which was frequently penicillin resistant.The early effect of PCV13 implementation greatly reduced the incidence of pneumococcal meningitis in children less than 15 years old. However, a sharp rebound in incidence linked to the emergence of serotype 24F compromised the long-term PCV efficacy. If confirmed in future studies and in other countries, pneumococcal meningitis incidence rebound and 24F emergence should be considered when developing next-generation PCVs.The French Pediatric Infectious Diseases Group, Association Clinique et Thérapeutique Infantile du Val de Marne, Pfizer, and for the National Reference Centre for Pneumococci, the French National Health Agency.

Low cost off-the-shelf depth sensors Microsoft Kinect have already been used in studies related to body condition determination and lameness detection in dairy cows and the concept of a 3D cow scanning by combining the fields of view of six Kinect cameras being presented. The cow scanner was developed in an effort to remove if possible, the influence of human operators from conformation recording by gathering data on linear descriptive traits using image analysis. In this study, a 3D object recognition pipeline was presented to automatically determine udder and rear leg of cows recorded in free walking. These body parts were then used to calculate the height of the udder bottom above ground and the rear leg angle. Between the manually gathered corresponding conformation recording scores and the calculated traits, medium to high Spearman rank correlation coefficients (0.63 for udder bottom height, 0.67 for rear leg angle) were observed. Between consecutive measurements, the calculating of udder bottom height was highly repeatable (76.9%) and the rear leg angle showed medium repeatability (47.4%) due to variance induced by the phase of the step. Both traits exhibited expected behaviour as udder bottom heights significantly decreased with increasing lactation number and rear legs were significantly straighter in cows with greater sacrum height.

Microsoft Kinect systems have already been used for detecting lameness and determining body condition in dairy cattle. A combination of six Kinect cameras was used with the goal of measuring linear descriptive traits. To access the precision of measurements gathered with a fixed installed recording unit, front teats and ischial tuberosities were marked manually in the recordings. Teat lengths and heights of ischial tuberosities were then calculated from the 3D coordinates. Recorded with cattle standing still and walking, teat lengths showed a standard error range from 0.7 mm to 1.5 mm and 1.8 mm–3.2 mm, respectively. The standard errors regarding the heights of the ischial tuberosities ranged between 2.4 mm and 4.0 mm in standstill and between 14.0 mm and 22.5 mm when measured on a walking cow.

Camera based systems in dairy cattle have been intensively studied over the last years. Past studies have concentrated on single camera systems with a limited range of applications. Here the development of a camera system comprising multiple 3D cameras (six Microsoft Kinect cameras) for monitoring purposes in dairy cows is presented. A recording unit was constructed, and software for recording, synchronising, sorting and segmenting images, and transforming the 3D data in a joint coordinate system was implemented. The latter is called extrinsic calibration and was dealt with in this study using a 3D calibration object. The internal estimations of acceleration data and coefficients for the parametrised plane describing the floor of the scenery obtained by the Kinect camera were tested and discussed with regard to their usage in the calibration process. The presented approach accurately determined whether a Kinect device was a side view camera or one of two types of top view cameras (accuracy 92.9%) and specified its mounting position in the system (accuracy 95.8%). Furthermore, rotations and translations between the cameras and a reference coordinate system were estimated from 3D point clouds of the calibration object. For cameras in upright position (side view cameras) the rotational accuracies were satisfactory, showing 4.4° (±2.9°) deviation from the reference. For the cameras in top view position larger errors (25.5° ± 6.4°) were measured due to them being mounted in an inclined way. Nevertheless, a basis for registration methods was achieved.

Background: There are no FDA approved therapies for neonatal seizures. Previous studies demonstrated that phenobarbital (PHB) and phenytoin frequently fail to control seizures. There are concerns about the safety of seizure medications in the developing brain. Levetiracetam (LEV) has proven efficacy in children and adults and has an excellent safety profile; therefore, there is great interest in its use in neonates, but randomized studies have not been performed. Methods: The study was a multicenter, randomized, blinded, controlled, phase IIb trial investigating the efficacy and safety of LEV compared with PHB in the first-line treatment of neonatal seizures, and including a dose escalation component. The primary outcome measure was complete seizure freedom for 24 hours after medication administration. Efficacy in seizure control was determined by independent review of the video EEGs by two neurophysiologists. Results: Eighty percent of subjects (24 of 30) randomized to PHB remained seizure free for 24 hours as compared with 28% of subjects (15 of 53) randomized to LEV (p

Camera-based systems in dairy cattle were intensively studied over the last years. Different from this study, single camera systems with a limited range of applications were presented, mostly using 2D cameras. This study presents current steps in the development of a camera system comprising multiple 3D cameras (six Microsoft Kinect cameras) for monitoring purposes in dairy cows. An early prototype was constructed, and alpha versions of software for recording, synchronizing, sorting and segmenting images and transforming the 3D data in a joint coordinate system have already been implemented. This study introduced the application of two-dimensional wavelet transforms as method for object recognition and surface analyses. The method was explained in detail, and four differently shaped wavelets were tested with respect to their reconstruction error concerning Kinect recorded depth maps from different camera positions. The images' high frequency parts reconstructed from wavelet decompositions using the haar and the biorthogonal 1.5 wavelet were statistically analyzed with regard to the effects of image fore- or background and of cows' or persons' surface. Furthermore, binary classifiers based on the local high frequencies have been implemented to decide whether a pixel belongs to the image foreground and if it was located on a cow or a person. Classifiers distinguishing between image regions showed high (⩾0.8) values of Area Under reciever operation characteristic Curve (AUC). The classifications due to species showed maximal AUC values of 0.69.

Resume Introduction Devant une importante frequentation du service d’accueil des urgences pediatriques (SAUP) du centre hospitalier universitaire (CHU) Lenval a Nice, il a ete instaure un systeme de circuit court avec des consultations delocalisees (CsD). L’objectif etait d’evaluer l’interet de sa mise en place en termes de duree d’attente et de prise en charge, de satisfaction des patients et des soignants. Methode Etude prospective, mono-centrique realisee en periode d’ouverture des CsD (le week-end de 13 h a 20 h) du 21 janvier au 18 mars 2012. Tous les enfants consultant au SAUP en periode d’ouverture des CsD pour raison medicale ont ete inclus. Parmi les enfants etiquetes stade 5, un sur deux etait transfere aux CsD. Les donnees evaluees ont ete les delais d’attente, la duree de consultation, le temps de passage et la satisfaction des patients et du personnel soignant. Resultats Un total de 875 patients a ete inclus. Les stades 1–2, 3, 4, 5 non delocalises et delocalises avaient respectivement des durees moyennes d’attentes entre la prise en charge par l’infirmiere d’accueil et la consultation medicale de 39,1 ; 57,7 ; 54,8 ; 70,5 et 52,7 minutes (min). La duree moyenne de consultation etait plus longue pour les stades 1–2 (61,6 min). Le temps global passe aux urgences etait plus long pour les stades 5 non delocalises (121,3 min). L’ensemble de la population des urgences (patients et equipe medicale) semblait satisfait par la mise en place des CsD. Conclusion Les delais d’attente et de prise en charge ont diminue pour les patients ayant beneficie des CsD.

To retrospectively evaluate the safety and efficacy of microwave ablation (MWA) as treatment for single, focal hepatic malignancies.Institutional review board approval was obtained for this HIPAA-compliant study. From December 2003 to May 2012, 64 patients were treated with MWA for a single hepatic lesion, in 64 sessions. Hepatocellular carcinoma (HCC) was treated in 25 patients (geometric mean tumor size, 3.33-cm; 95% CI, 2.65-4.18-cm; range, 1.0-12.0-cm), metastatic colorectal cancer (CRC) was treated in 27 patients (geometric mean tumor size, 2.7-cm; 95% CI, 2.20-3.40-cm; range, 0.8-6.0-cm), and other histological-types were treated in 12 patients (geometric mean tumor size, 3.79-cm; 95% CI, 2.72-5.26-cm; range, 1.7-8.0-cm). Kaplan-Meier (K-M) method was used to analyze time event data. Chi-square and correlation evaluated the relationship between tumor size and treatment parameters.Technical success rate was 95.3% (61/64). Treatment parameters were tailored to tumor size; as size increased more antennae were used (p0.001), treatment with multiple activations increased (p0.028), and treatment time increased (p0.001). There was no statistically significant relationship between time to recurrence and tumor size, number of activations, number of antennae, and treatment time. At one-year, K-M analysis predicted a likelihood of local recurrence of 39.8% in HCC patients, 45.7% in CRC metastases patients, and 70.8% in patients with other metastases. Median cancer specific survivals for patients were 38.3 months for HCC patients, 36.3 months for CRC metastases, and 13.9 months for other histological-types. Complications occurred in 23.4% (15/64) of sessions.In our sample, tumor size did not appear to impact complete ablation rates or local recurrence rates for focal hepatic malignancies treated with MWA.

Resume Bien que des maladies graves puissent etre transmises par les moustiques, les manifestations les plus frequentes sont essentiellement liees a la reaction au moment du contact avec la salive des moustiques lors du repas sanguin. Ce sont surtout Culex et Aedes que l’on rencontre dans les pays a climats temperes. Les manifestations cliniques vont varier en fonction de la reponse immunoallergique de l’individu, allant de simples papules prurigineuses a des reactions allergiques immediates et/ou retardees parfois severes. Certaines reactions peuvent provoquer une confusion avec des dermohypodermites infectieuses. L’evolution naturelle se fait en cinq stades au fur et a mesure que l’individu est pique par les moustiques jusqu’a ce qu’une tolerance immunitaire s’installe. Les tests allergiques cutanes ou seriques a base d’extraits de corps entier de moustique manquent de sensibilite et de specificite. Ils sont reserves aux cas les plus invalidants ou severes. L’identification recente des allergenes principaux de la salive des moustiques et l’obtention d’allergenes recombinants devraient permettre d’ameliorer le diagnostic et d’envisager une immunotherapie.

Increasing milk production per cow over the last decades has led to a more intense negative energy balance and extended the duration of body fat mobilization. Management of body condition requires that body condition scores be evaluated on a visual basis. A more objective approach is the measurement of the backfat thickness (BFT) using an ultrasound device. Metering precision of 1 mm enables the recognition of even slight changes in the subcutaneous fat thickness. Because both methods are time consuming in large dairy herds, automating body condition׳s routine evaluation is of rising concern. This study investigated whether traits of the animals׳ rear area extracted from Time-of-Flight camera recordings might be useful indicators of BFT. Furthermore, characteristics of BFT within and across different lactation stages were analyzed. Data recording was performed on the dairy research farm Karkendamm of the Institute of Animal Breeding and Husbandry of the University of Kiel (Germany) from July 2011 to May 2012. BFT measures ( n =2931) and two measures ( n =1779 and n =1848) obtained from Time-of-Flight recordings from 96 primiparous and multiparous Holstein-Friesian dairy cows were analyzed using seven different linear (mixed) models. BFT estimation using the cow as random or fixed effect, lactation week and observation month as fixed effects, and a linear regression on the two Time-of-Flight measurements has been promising. The correlation among observed values and estimator was 0.96. Repeatability of BFT within each of the three lactation stages was large and ranged from 0.80 to 0.89. Correlations of cow effects among lactation stages were also high and range from 0.65 between first and third lactation stage to 0.89 between second and third lactation stage.

Mutations in POMT1 lead to a group of neuromuscular conditions ranging in severity from Walker-Warburg syndrome to limb girdle muscular dystrophy. We report two male siblings, ages 19 and 14, and an unrelated 6-year old female with early onset muscular dystrophy and intellectual disability with minimal structural brain anomalies and no ocular abnormalities. Compound heterozygous mutations in POMT1 were identified including a previously reported nonsense mutation (c.2167dupG; p.Asp723Glyfs*8) associated with Walker-Warburg syndrome and a novel missense mutation in a highly conserved region of the protein O-mannosyltransferase 1 protein (c.1958C>T; p.Pro653Leu). This novel variant reduces the phenotypic severity compared to patients with homozygous c.2167dupG mutations or compound heterozygous patients with a c.2167dupG mutation and a wide range of other mutant POMT1 alleles.

Resume Les infections du site operatoire (ISO) sont une cause importante de mortalite et de morbidite postoperatoire de meme qu’elles engendrent un surcout financier. Parmi les mesures de prevention, l’antibioprophylaxie diminue significativement ce risque. Des recommandations recentes ont ete publiees chez l’adulte. Bien que les donnees pediatriques soient rares, ces recommandations peuvent etre majoritairement extrapolees a l’enfant a l’exception de la periode neonatale. Pour les interventions justifiant d’une antibioprophylaxie, la molecule de choix est une cephalosporine de premiere generation non utilisee en curatif comme la cefazoline avec un ajustement de posologie. Une injection peroperatoire est generalement suffisante et ne doit pas depasser 24 heures sauf situation exceptionnelle.

The French Group of Pediatric Infectious Diseases (PID) of the French Society of Pediatrics found necessary to issue in 2011 therapeutic proposals concerning antibiotic treatment in severe ENT infections in children (acute mastoiditis, severe sinusitis, peripharyngeal abscess). They took into account, for each clinical situation, published studies and existing guidelines, the most frequently encountered bacterial species, their usual sensitivity to antibiotics, their pharmacokinetic and pharmacodynamic (PK-PD) characteristics. These propositions aim to ensure the proper use of antibiotics and to limit the development of bacterial resistance to antibiotics by minimizing the use of broadspectrum molecules, especially cephalosporins and penems. These infections are often multi microbial and respond to aerobic flora similar to that found in non severe community acquired ENT infections and soft tissue infections ( Streptococcus pyogenes or group A Streptococcus(GAS), Streptococcus pneumoniae, Staphylococcus aureus and Haemophilus influenzae). Anaerobic flora may be associated and implicated in some situations. In most cases, high-dosage of amoxicillin +/- clavulanic acid offers the best PK/PD profile and allows to avoid the overuse of injectable third-generation cephalosporins.

Resume L’infection est la cause la plus frequente de morbidite et de mortalite chez les enfants immunodeprimes. L’emergence des bacteries resistantes est particulierement inquietante pour cette population. Le risque infectieux peut etre diminue par des regles d’education, une vaccination adaptee et parfois une antibioprophylaxie. Cependant, le risque infectieux reel est difficile a evaluer a l’echelon individuel, et l’utilisation prolongee et repetee d’antibiotiques peut etre deletere : reactions allergiques, interactions medicamenteuses, surinfections, colite pseudomembraneuse et surtout developpement de resistance bacterienne. Des recommandations emanant de societes savantes existent notamment en ce qui concerne les deficits immunitaires hereditaires et l’allogreffe de cellules souches hematopoietiques. Dans d’autres cas, cela depend des habitudes de chacun : l’evolution de la resistance aux antibiotiques doit conduire a reduire au maximum ces antibiotherapies non fondees sur des preuves ou des recommandations. Le developpement de nouveaux vaccins notamment antipneumococciques et antimeningococciques pourrait changer les recommandations et les habitudes.

Resume La mastoidite aigue est une complication classique mais rare de l’otite moyenne aigue purulente. La prise en charge d’une mastoidite est medicale avec une antibiotherapie initiale intraveineuse et eventuellement chirurgicale pour les formes compliquees. L’adequation de l’antibiotherapie probabiliste initiale avec les germes potentiellement impliques est essentielle pour eviter les complications potentiellement graves. Cette revue de la litterature a comme but de fournir les arguments qui soutiennent les propositions therapeutiques du Groupe de pathologie infectieuse pediatrique concernant l’antibiotherapie de premiere intention des mastoidites de l’enfant.

Resume Les cellulites ou abces intra- ou extra-orbitaires d’origine bacterienne sont les complications les plus frequentes des sinusites aigues. Les bacteries responsables sont Streptococcus pneumoniae , Streptococcus pyogenes , Staphylococcus aureus (SA) et les bacteries anaerobies, et beaucoup plus rarement Haemophilus infl uenzae (surtout depuis la vaccination contre le type b). Compte tenu de la sensibilite de ces bacteries et des parametres PK/PD des antibiotiques, et en fonction de la classifi cation de Chandler, plusieurs schemas therapeutiques probabilistes sont proposes : dans la classe 1 (cellulite preorbitaire), amoxicilline-acide clavulanique per os (80 mg/kg/24 h d’amoxicilline en 2 doses) ou ceftriaxone en IM ; et dans les classes 2 a 5, amoxicilline-acide clavulanique a fortes doses en intraveineux (jusqu’a 150 mg/kg/j d’amoxicilline), ou l’association intraveineuse de cefotaxime (200 mg/kg/j) ou ceftriaxone (100 mg/kg/j) a un anti-anaerobie type metronidazole (30 mg/kg/j) ou clindamycine (40 mg/kg/j).

Neck infections in children are categorized as peritonsillar infections, latero and retroparapharyngeal infections. The clinical features and severity of these infections vary according to different paediatric age groups, in relation to the location of the infection. In France, the antimicrobial therapy should consider meticillin sensitive S. aureus, Streptococcus pyogenes and anerobic bacteria. Empiric initial antimicrobial therapy consists in high doses of parenteral amoxicillin-clavulanate (150 mg/kg d. in 3-4 doses) during a few days then changed to oral amoxicillin-clavulanate (80 mg/kg/d). The total course of therapy should be 10 to 14 days.

To evaluate the potential importance in autistic subjects of copy number variants (CNVs) that alter genes of relevance to bioenergetics, ionic metabolism, and synaptic function, we conducted a detailed microarray analysis of 69 autism probands and 35 parents, compared to 89 CEU HapMap controls. This revealed that the frequency CNVs of≥100kb and CNVs of≥10 Kb were markedly increased in probands over parents and in probands and parents over controls. Evaluation of CNVs≥1Mb by chromosomal FISH confirmed the molecular identity of a subset of the CNVs, some of which were associated with chromosomal rearrangements. In a number of the cases, CNVs were found to alter the copy number of genes that are important in mitochondrial oxidative phosphorylation (OXPHOS), ion and especially calcium transport, and synaptic structure. Hence, autism might result from alterations in multiple bioenergetic and metabolic genes required for mental function. This article is part of a Special Issue entitled: 17th European Bioenergetics Conference (EBEC 2012).

The E-pathway of transmembrane proton transfer has been demonstrated previously to be essential for catalysis by the diheme-containing quinol:fumarate reductase (QFR) of Wolinella succinogenes. Two constituents of this pathway, Glu-C180 and heme b(D) ring C (b(D)-C-) propionate, have been validated experimentally. Here, we identify further constituents of the E-pathway by analysis of molecular dynamics simulations. The redox state of heme groups has a crucial effect on the connectivity patterns of mobile internal water molecules that can transiently support proton transfer from the b(D)-C-propionate to Glu-C180. The short H-bonding paths formed in the reduced states can lead to high proton conduction rates and thus provide a plausible explanation for the required opening of the E-pathway in reduced QFR. We found evidence that the b(D)-C-propionate group is the previously postulated branching point connecting proton transfer to the E-pathway from the quinol-oxidation site via interactions with the heme b(D) ligand His-C44. An essential functional role of His-C44 is supported experimentally by site-directed mutagenesis resulting in its replacement with Glu. Although the H44E variant enzyme retains both heme groups, it is unable to catalyze quinol oxidation. All results obtained are relevant to the QFR enzymes from the human pathogens Campylobacter jejuni and Helicobacter pylori.

Resume La reunion annuelle de la Societe americaine d’infectiologie (Infectious Disease Society of America [IDSA]), qui a reuni pres de 5000 congressistes de plus de 80 pays a Vancouver, Canada du 21 au 24 octobre 2010, a permis de faire le bilan de la pandemie de grippe A(H1N1) 2009 en Amerique du Nord, d’evaluer les programmes de vaccination, et de presenter les nouveaux vaccins en cours de developpement. Avec 12 500 deces aux Etats-Unis en 2009–2010, la grippe pandemique a ete moins meurtriere que la grippe saisonniere. Mais elle a frappe essentiellement des jeunes, et le bilan calcule en nombre d’annees de vie perdues est eleve. Les differents vaccins monovalents (vivants attenues, inactives avec ou sans adjuvants) ont ete bien toleres, chez les enfants comme chez les adultes et les femmes enceintes. La protection des tres jeunes nourrissons contre la coqueluche passe par le renforcement des rappels vaccinaux de son entourage familial. L’introduction du vaccin pneumococcique conjugue 13-valent au debut de l’annee 2010 pourrait resoudre – mais pour combien de temps? – le probleme de remplacement des serotypes, responsable d’une recrudescence des infections invasives a pneumocoques observee dans les pays qui avaient introduit le vaccin conjugue 7-valent. L’efficacite du vaccin rotavirus se confirme, avec une diminution des hospitalisations aux Etats-Unis et une diminution des deces dus aux gastroenterites au Mexique. Aux Etats-Unis, la vaccination des preadolescentes contre le papillomavirus humain (HPV) n’a pas entraine d’effet indesirable particulier. La vaccination de routine contre la varicelle, recommandee depuis 1995, n’a eu aucun impact sur l’evolution de l’incidence du zona. Quant au vaccin contre le zona, recommande pour les sujets de 60 ans et plus aux Etats-Unis, son efficacite sur le terrain serait de 55 %, d’apres une etude de cohorte (Kaiser Permanente de Californie du Sud). Si certains proposent l’elaboration de vaccins a la carte, en fonction des caracteristiques genetiques individuelles, la priorite reste l’augmentation de la couverture vaccinale, non seulement chez les enfants, mais aussi chez les adultes et les personnes âgees. Il convient de proposer un calendrier vaccinal etale sur toute la vie, la vaccination a l’âge adulte et chez les seniors etant determinante pour une bonne sante a tous les âges de la vie.

The ITER torus and neutral beam cryopumps are complex vacuum pumps which will be designed to the build-to-print level stage for the call for tender for manufacturing. Although being completely different in size, the pumps share the same concept of cryosorption on activated charcoal. Thus, the design procedure itself can follow the same generic workflow. The requirements on the pumps are summarized and the status of design evolution is described. Both design packages require specific R&D for experimental clarification and design validation. Two examples for this approach are given, namely the characterization of the closing forces of the torus pump inlet valve, and the measurement of hydraulic resistance coefficients of hydroformed components.

Purpose: The diagnosis of a mitochondrial disorder relies heavily on the enzymatic analysis of mitochondrial respiratory chain complexes in muscle or other tissues. However, considerable differences exist between clinical laboratories in the protocols or particular tests used for evaluation. In addition, laboratories can encounter difficulties in consistent technique, as well as procurement of adequate positive or negative controls. Currently, there is no external quality assurance for respiratory chain complex assays. In this study, we explored the use of Caenorhabditis elegans mitochondria as a potential aid to diagnostic centers that perform respiratory chain complex assays. Method: Five diagnostic test centers in the United States and one from Australia comparatively analyzed enzyme activities of mitochondria from C. elegans. The first survey consisted of three open-labeled samples including one normal control and two mutants; the second survey consisted of one open-labeled normal control and two blinded samples. Results: There was very good concordance among laboratories in detecting the majority of the defects present in the mutant specimens. Despite the ability to detect respiratory chain complex defects, the scatter between centers for certain enzymatic assays, particularly I + III, II, III, and IV, led to different diagnostic interpretations between the centers. Conclusion: The data strongly support the need for comparative testing of mitochondrial enzyme assays between multiple laboratories. Our overall results are encouraging for the use of nematode mitochondria as a tool that might provide a virtually inexhaustible supply of mitochondria with defined defects for development of assays and as a potential source of control specimens.

We studied cytochrome c oxidase (COX) expression patterns in nuclear and mtDNA gene defects. Using quantitative immunocytochemical assay for COX, heteroplasmic staining was seen in MELAS patients with mtDNA mutations but similar staining variability was seen in control cell lines and nuclear gene defects. All fibroblast lines showed a wide variability in cell-to-cell COX I staining intensity. All 8 patient fibroblast lines had reduced COX staining on immunocytochemistry. In 6 lines reduced protein amount was seen on Western blotting and 7 had low COX activity. This study demonstrates that nuclear gene defects can produce a heteroplasmic appearance on immunocytochemistry.

Medium-chain Acyl-CoA dehydrogenase deficiency (MCAD) is one of the most common fatty acid oxidation disorders. Clinical manifestations can be serious and lead to death if unrecognized. They are not specific and can mimic meningitis or an acute intestinal intussusception in its neurological form. Early recognition of MCAD and presymptomatic treatment of intercurrent illness improve the prognosis over the short- and long-term. MCAD deficiency satisfies the major criteria for newborn screening. We report the cases of 2 patients whose presentation was typical and severe. Early diagnosis of MCAD deficiency helped to start a simple treatment in both patients aimed at preventing further decompensation.

The APW + lo (augmented plane waves + local orbitals) method of density functional theory, as implemented in the Wien2k code, is applied to calculate the electric field gradient of manganites. We report the first principles calculations on CaMnO 3 , and modeling of a perturbed angular correlation experiment with implanted Cd 111 m isotope. To model the experiment we additionally calculate in Ca 1 - x Cd x MnO 3 , where we substitute Cd at the Ca site. Increasing Cd dilution is done with the use of supercells. We find that the experimental CaMnO 3 low-temperature value V zz ≈ 6 × 10 - 21 V / m 2 is reproduced, when optimizing internal parameters. The analysis of the EFG tensor at the Ca atoms, with different electric field gradients at inequivalent positions, reveals that the convergence of the calculations is obtained.

Leishmaniases are parasitic diseases due to a flagellate protozoan of the genus Leishmania. They are transmitted from mammal to mammal by the bite of an arthropod vector: a female sandfly. Among the different clinical presentations, the zoonotic visceral leishmaniasis (ZVL) is due to Leishmania infantum. Dogs are the reservoir and can develop a deadly disease. ZVL is described in China, Pakistan, Latin America and in the Mediterranean region, particularly in the South of France. In recent years, many asymptomatic carriers have been described. Despite the fact that cases in immunocompromised adults are the majority, the classic Mediterranean ZVL in young children is still observed. The classic triad of symptoms is: fever, pallor, splenomegaly and hepatomegaly in half of the cases. The biological orientation is a low blood count (anemia, leuconeutropenia, and thrombocytopenia) and an inflammatory syndrome. Serological tests are useful, but the diagnosis is made by the identification of the parasite in a bone marrow sample. Today, the treatment is done by the liposomal amphotericin B (AmBisome) and the total dose must to be 20 mg/kg.

Chikungunya fever is an arbovirosis caused by an alphavirus (CHIKV) belonging to the Togaviridae family. Its main vectors are Aedes mosquitoes. In its classic form, Chikungunya consists in a flu-like illness that can be very disabling, especially by incapacitating arthralgia. In children, the arthropathy is well known to be better tolerated than in adulthood but severe manifestations and complications can occur owing to neurologic, cardiac, hematologic or cutaneous dysfunctions, all carrying a fatality risk in the absence of appropriate intensive care. Out of these, the most singular is a severe encephalopathy, even in some cases genuine encephalitis. More rare, but quite specific of small infants, skin blisters have been reported, sometimes complicated by extensive detachments. Mother-to-child infections were demonstrated on La Reunion island with a fifty-percent probability of vertical transmission when the mother was highly viremic around the term of pregnancy. The diagnosis can be made by detecting CHIKV RNA using RT-PCR or specific IgM antibodies using MAC-Elisa serology. Chikungunya is a notifiable disease. The epidemic that emerged in Indian Ocean islands during 2005-2006, its progressive extension to Asia and even to Italy in July 2007, highlighted a very important capacity of CHIKV to cause huge outbreaks wherever Aedes sp. can proliferate. In France, Aedes albopictus is definitively endemic in the departments of Alpes-Maritimes since 2004, Corsica since 2005, and Var since 2007. Therefore, the risk of introduction of CHIKV from an epidemic area to Europe and especially in France is real.

Visceral leishmaniasis (VL) causes an estimated 500,000 new cases of disease and more than 50,000 deaths a year. For more than 60 years, pentavalent antimonies were considered the standard therapy for VL. The emergence of Leishmania strains resistant to antimonials led to the evaluation of other treatments including amphotericin B and its lipidic derivatives. Clinical trials with liposomal amphotericin B demonstrated that total doses of 10 to 20mg/kg, administered according to various regimens, had a 90-98% efficacy in non-immunocompromised patients. Compared to antimonials, liposomal amphotericin B provides favorable efficacy/tolerance and cost efficacy ratios. The WHO recently produced consensus recommendations for the use of liposomal amphotericin B in VL. In Europe, liposomal amphotericin B has progressively become the reference treatment of VL in clinical practice, and it is recommended as the first line therapy.

The ITER sorption cryopumps will see different regeneration stages, one of them being a high temperature regeneration step to release quantitatively all water-like substances with strong sorption bonding forces to the activated charcoal sorbent used in the pumps. Tests have been made in the TIMO test bed at Forschungszentrum Karlsruhe with a model of the ITER torus cryopump to investigate the conditions needed to meet this requirement. It is shown that 470 K is a ‘one for all’ temperature level believed to regenerate from all species expected to be seen from the ITER cryopumps, including hydrocarbons. For only water regeneration, which will be the most important case, the establishment of low pressures at up to maximum 400 K is an alternative option.

Guanidinoacetate methyltransferase (GAMT) deficiency is a disorder of creatine biosynthesis, characterized by excessive amounts of guanidinoacetate in body fluids, deficiency of creatine in the brain, and presence of mutations in the GAMT gene. We present here 8 new patients with GAMT deficiency along with their clinical, biochemical and molecular data. The age at diagnosis of our patients ranges from 0 to 14 years. The age of onset of seizures usually ranges from infancy to 3 years. However, one of our patients developed seizures at age 5; progressing to myoclonic epilepsy at age 8 years and another patient has not developed seizures at age 17 years. Five novel mutations were identified: c.37ins26 (p.G13PfsX38), c.403G>T (p.D135Y), c.507_521dup15 (p.C169_S173dup), c.402C>G (p.Y134X) and c.610_611delAGinsGAA (p.R204EfsX63). Six patients had the c.327G>A (last nucleotide of exon 2) splice-site mutation which suggests that this is one of the most common mutations in the GAMT gene, second only to the known Portuguese founder mutation, c.59G>C (p.W20S). Our data suggests that the clinical presentation can be variable and the diagnosis may be overlooked due to unawareness of this disorder. Therefore, GAMT deficiency should be considered in the differential diagnosis of progressive myoclonic epilepsy as well as in unexplained developmental delay or regression with dystonia, even if the patient has no history of seizures. As more patients are reported, the prevalence of GAMT deficiency will become known and guidelines for prenatal diagnosis, newborn screening, presymptomatic testing and treatment, will need to be formulated.

ResumeObjectifs Evaluer la charge de travail medical et infirmier induite par les gastroenterites aigues (GEA) en periode epidemique. Methodes Etude observationnelle, multicentrique, menee dans 23 services d’urgences pediatriques francais. Chaque service devait inclure les 25 premiers enfants (

The empiric choice of initial antibiotherapy in osteoarticular infections in infants and children must take into consideration the actual epidemiology of principal pathogens, their respective antibiotic sensitivity profile, their pharmacokinetic and pharmacodynamic properties and the results of efficacy clinical studies. After a review of recent data concerning these four major points, the Paediatric Infectious Diseases Group of the French Society of Paediatrics (GPIP) has proposed guidelines for initial recommended schemes of antimicrobial therapy in acute and non complicated osteoarticular infections in infants and children.

Management of paediatric skeletal infections remains delicate in France due to the absence of general agreement or official recommendations from the French National Societies. However, practices have evolved since 40 years towards simplified and mostly ambulatory treatment modalities. The Paediatric Infectious Diseases Group of the French Society of Paediatrics (GPIP) has elaborated guidelines funded on the joint analysis of bacterial epidemiology, comparison of common antimicrobial spectra, pharmacokinetic and pharmacodynamic parameters and clinical studies. The aim of this article is to review the evolution of therapeutic concepts of treatment of bone and joint infections in paediatrics for 40 years with the aid of main published clinical studies.

Resume Objectif Evaluer l’influence du test de diagnostic rapide de la grippe (TDRG) sur la prescription d’examens complementaires, d’antibiotiques et d’oseltamivir ainsi que l’impact de la prescription d’oseltamivir sur les arrets de travail et la duree des symptomes cliniques. Methodes Etude prospective aux urgences pediatriques du CHU de Nice du 29 janvier 2007 au 05 mars 2007, incluant les enfants de 1 mois a 6 ans avec fievre superieure ou egale a 38,5 °C depuis moins de 48 h. La recherche virologique pratiquee sur aspiration nasopharyngee a comporte : une immunofluorescence, une culture et un TDRG Quickvue ® . Les donnees cliniques, examens complementaires et traitements etaient enregistres pour chaque enfant. Un traitement antiviral (oseltamivir) etait propose chez les enfants TDRG positif et âges de plus de 1 an. L’evolution a 7 j a ete evaluee par contact telephonique. Resultats Cent soixante-dix-sept enfants ont ete inclus (âge moyen 24 mois, sex-ratio 1,88). Le TDRG etait positif dans 42,3 % des cas ( n = 75). Par rapport a la culture, le TDRG presentait une sensibilite, specificite, valeur predictive positive et valeur predictive negative respectivement de 95,6, 91,6, 88 et 97 %. Les signes cliniques significativement correles a la grippe etaient l’alteration de l’etat general, la rhinorrhee et l’otite moyenne aigue. Dans le groupe avec TDRG positif, il y avait de maniere significative moins de prescription d’examens complementaires (13 versus 36) et particulierement d’examen cytobacteriologique des urines (ECBU) (5 versus 19), et plus de dissemination dans l’entourage ( p = 0,0002). Il n’y avait pas de difference significative en terme d’hospitalisation, de prescription d’antibiotiques ni d’arrets de travail des parents. Conclusion Le TDRG dans un service d’urgences en periode epidemique permet une diminution d’examens complementaires notamment d’ECBU.

Denaturing high-performance liquid chromatography (DHPLC) purification of heteroduplexes has been reported as a method to increase sensitivity of the detection of low-level heteroplasmy by DNA sequencing, and DHPLC profiling has been suggested as a method to allow the correlation of a characteristic chromatographic profile with a specific sequence alteration. Herein we report pitfalls associated with the use of DHPLC for these purposes. We show that the purified heteroduplex fraction does not contain a 50:50 mix of wild-type and mutant DNA in DNA samples containing low-level mutations, and that with a commonly used protocol, DNA sequencing gave false negative results at the 1% mutation level, potentially leading to misdiagnosis. We improved the protocol to detect low levels of mutations and evaluated the sensitivity of DNA sequencing in the detection of mutation in these fractions. We also studied the DHPLC profiles of several mutations in the tRNALeu(UUR) region of mitochondrial DNA and found a characteristic profile in only one of five mutants tested, whereas four other mutants showed identical chromatographic profiles.

Forschungszentrum Karlsruhe (FZK) is developing the ITER prototype torus exhaust pumping system, comprising eight identical cryosorption pumps to provide a high pumping speed and capacity, and fast regeneration. The prototype pump features an integral inlet valve with a nominal diameter of 800 mm which can be closed during the plasma pulse operation for on-line regeneration. The pump design is incorporating the results of the experimental campaigns performed with the model torus cryopump in the TIMO test bed at FZK as well as the results achieved in the large scale tritium testing of an ITER relevant cryosorption pump set-up at JET. This paper summarizes the requirements for the pumping system and outlines how these are successfully met. The way in which the nominal parameters for the detailed design of the cryopumps are derived from the overall system specification is described. Finally, the detailed design of the pump and its sub-assemblies will be presented.

The evidence supporting a treatment benefit for coenzyme Q10 (CoQ10) in primary mitochondrial disease (mitochondrial disease) whilst positive is limited. Mitochondrial disease in this context is defined as genetic disease causing an impairment in mitochondrial oxidative phosphorylation (OXPHOS). There are no treatment trials achieving the highest Level I evidence designation. Reasons for this include the relative rarity of mitochondrial disease, the heterogeneity of mitochondrial disease, the natural cofactor status and easy 'over the counter availability' of CoQ10 all of which make funding for the necessary large blinded clinical trials unlikely. At this time the best evidence for efficacy comes from controlled trials in common cardiovascular and neurodegenerative diseases with mitochondrial and OXPHOS dysfunction the etiology of which is most likely multifactorial with environmental factors playing on a background of genetic predisposition. There remain questions about dosing, bioavailability, tissue penetration and intracellular distribution of orally administered CoQ10, a compound which is endogenously produced within the mitochondria of all cells. In some mitochondrial diseases and other commoner disorders such as cardiac disease and Parkinson's disease low mitochondrial or tissue levels of CoQ10 have been demonstrated providing an obvious rationale for supplementation. This paper discusses the current state of the evidence supporting the use of CoQ10 in mitochondrial disease.

The next generation thermonuclear fusion machine ITER comprises large cryogenic pumping systems to process very high gas throughputs. The paper starts from a summary of the requirements for the torus exhaust pumping system and the neutral beam injection system for plasma heating and discusses the design solutions derived to match them. All ITER force-cooled cryopumps are based on a modular set-up and incorporate similar design of charcoal-coated cryopanels cooled to 4.5 K with supercritical helium. A systematic approach for how to design a tailor-made cryopump is presented. It is based on Monte Carlo simulation to calculate the overall capture coefficient being the primary design parameter. Experimental results measured with a model pump of representative scale are used to validate the design procedure. In the last part, the paper addresses the design issues to be solved for tritium-compatible forepumps with high pumping speeds.

Resume Objectif . – Decrire les differentes modalites de prise en charge de l'invagination intestinale aigue (IIA) du nourrisson et de l'enfant en France et identifier les centres susceptibles de participer a un eventuel reseau de surveillance de l'IIA en France a l'arrivee des nouveaux vaccins rotavirus. Materiel et methodes . – Enquete effectuee aupres des medecins et chirurgiens referents de 273 centres d'accueil d'urgences pediatriques repartis sur le territoire national. Le questionnaire portait sur les modalites de prise en charge diagnostique et therapeutique de l'IIA, en 2005. Resultats . – Cent soixante-sept reponses (61,2 %) ont ete obtenues, provenant de 131 pediatres (78,4 %) et 36 chirurgiens (21,6 %) repartis sur 38 CHU (22,7 %) et 129 CHG (77,2 %). La moyenne annuelle declaree d'IIA par centre est de 11 ± 13,5 (extremes : 0 a 70 ; mediane : 6). La prise en charge diagnostique mobilise les deux equipes medicale et chirurgicale dans 51,5 % des cas et la seule equipe medicale dans 40,1 % des cas. Dans 98,8 % des centres, l'echographie est pratiquee d'emblee. La reduction de l'IIA par lavement, suivie eventuellement de chirurgie est possible sur place dans 44,3 % des centres. Dans les autres, les transferts pour chirurgie sont effectues dans 90 % des cas vers un CHU. Conclusion . – Si la prise en charge des IIA en France est globalement homogene en ce qui concerne les methodes diagnostiques, les circuits de prise en charge therapeutique varient selon la disponibilite sur place d'equipes chirurgicales et anesthesistes experimentees en pediatrie. Ces disparites seront amenees a evoluer encore avec la mise en application du schema regional d'organisation sanitaire (SROS) de l'enfant et de l'adolescent de 2004. A terme, les services labellises pour les urgences pediatriques concentreront de plus en plus les patients chirurgicaux. Ces services pourraient former la trame d'un eventuel reseau hospitalier de surveillance de l'IIA en France.

The ITER in-vessel hydrogen inventory is highly dynamic and determined by a broad range of parameters. Moreover, it is of prime importance to allocate the inventory as accurately as possible. Two essential control issues have to be met. The basic safety approach is to limit the amount of hydrogen such that the resulting pressure in case of an explosion accident is compatible to the design criteria of the vacuum vessel. This requirement drives the operational philosophy and the design of the cryogenic pumps which are connected to the vessel. The administrative limitation of the tritium inventory is another essential requirement on reactor operation. This paper illustrates how hydrogen inventory control issues are reflected by the operational schemes of the vacuum pumping systems. Latest results include the release characteristics of hydrogens and tritium during normal regeneration of the cryopumps and under off-normal events. The different semi-permanent contributions to the overall tritium inventory are discussed. The forepumping systems reduce the in-vessel inventory and act as interface to the tritium plant. A typical example of an integral pump-out curve is discussed. It is shown that sufficient safety margin is included in the ITER vacuum pumping design.

Within the European research activities for the ITER fusion reactor, at the Forschungszentrum Karlsruhe (FZK) a test bed for the half-scaled ITER cryosorption pump was installed to study all the operational modes of interest. Following an extensive test program under ITER-like throughput conditions, additional studies were performed to assess the influence of liquid impurities on the sorption behaviour. After an extensive test program could already be successfully carried out the influences of liquid impurities on the pump performance should be studied. Tests with both water-like impurities and different tracer candidates were used during ITER-relevant operation cycles. Independent of the different tested impurities it could be proved that all requirements for the primary ITER torus vacuum pumps can be fulfilled with the actual vacuum pump concept. The total test program demonstrated that the adopted design concept meets all the ITER pumping requirements.

Resume La varicelle est une maladie contagieuse de la petite enfance souvent consideree comme benigne. Cependant, dans un certain nombre de cas, cette maladie peut presenter des formes beaucoup plus serieuses ou entrainer des complications parfois mortelles, notamment chez l’adulte, le nouveau ne, la femme enceinte et les patients immunodeficitaires. Les antiviraux, et particulierement l’acyclovir, apportent une possibilite therapeutique importante a condition d’etre debutee dans les 24 premieres heures apres l’apparition des vesicules. Les autres traitements tels que le valacyclovir, le famcyclovir et la brivudine sont discutes. Bien que plusieurs resultats soient encourageants, la place de l’acyclovir en prophylaxie de la maladie reste a preciser. Dans les situations d’immunodepression, il convient d’etre attentif aux formes de presentations atypiques ou prolongees, pouvant traduire l’existence d’une resistance virale a l’acyclovir. Cette situation fera alors discuter les alternatives therapeutiques que sont le foscarnet et le cidofovir en fonction du type de resistance mise en evidence apres etude virologique phenotypique et genotypique.

Resume Avant l'annonce officielle des recommandations sur la vaccination de la varicelle en France et en prevision de la mise a disponibilite d'un vaccin, des enquetes ont ete menees aupres de pediatres et du grand public pour evaluer leur perception de la maladie et l'interet de la vaccination dans le cadre d'une vaccination generalisee des nourrissons. Ces enquetes realisees entre septembre et novembre 2003 montrent que la varicelle est percue par les meres comme etant une maladie presentant des risques de complications et elles souhaitent la vaccination de leurs enfants a une large majorite (86 %). Les medecins jouent un role primordial dans l'information et les conseils a apporter aux meres et accordent eux-memes une place tres favorable a cette vaccination (92,8 % d'intention de vaccination si demande maternelle). Ces enquetes d'acceptabilite viennent en complement de donnees epidemiologiques de surveillance qui seront utiles a la revision des recommandations vaccinales actuelles.

In this review we will focus on the recent advances in how coiled-coil proteins of the golgin family give identity and structure to the Golgi apparatus in animal cells. A number of recent studies reveal a common theme for the targeting of golgins containing the ARL-binding GRIP domain, and the related ARF-binding GRAB domain. Similarly, other golgins such as the vesicle tethering factor p115 and Bicaudal-D are targeted by the Rab GTPases, Rab1 and Rab6, respectively. Together golgins and their regulatory GTPases form a complex network, commonly known as the Golgi matrix, which organizes Golgi membranes and regulates membrane trafficking.