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Introduction: Many critically ill children can be fed orally at some point during their paediatric intensive care (PICU) stay, but reduced appetite and other factors may impact on their intake. At home, oral feeding is usually delivered by parents; so involving parents more actively during the mealtimes in PICU may contribute to improved patient/family satisfaction. We aimed to assess the impact of a new “room service” initiative involving parents on mealtime quality and on both family and healthcare professional (HCP) satisfaction.\ud Methods: A prospective, single centre, before and after intervention study was designed, as part of a PICU quality of care improvement program in 2013-2016. Two questionnaires assessing oral nutrition practices and family/HCP overall satisfaction were disseminated among the parents of critically ill children capable of oral feeding during their PICU admission and among the whole PICU healthcare professional team (nurses, nurse assistants, and medical doctors). Categorical variables were compared using the chi-square test and Likert scales were compared between groups with the Mann-Whitney-Wilcoxon test.\ud Results: the pre-intervention surveys were completed by 97/130 (75%) HCPs and 52 families, and the post-intervention surveys by 74/130 (57%) HCPs and 54 families. After the intervention, a marked improvement was shown for the overall quality of meal service rating by both HCPs and families (medians and IQR: 5 (5-7) to 7 (7-8) and 6 (6-8) to 8 (7-9) respectively; p

The forthcoming SOXS (Son Of X-Shooter) will be a new spectroscopic facility for the ESO New Technology Telescope in La Silla, focused on transient events and able to cover both the UV-VIS and NIR bands. The instrument passed the Final Design Review in 2018 and is currently in manufacturing and integration phase. This paper is focused on the assembly and testing of the instrument control electronics, which will manage all the motorized functions, alarms, sensors, and electric interlocks. The electronics is hosted in two main control cabinets, divided in several subracks that are assembled to ensure easy accessibility and transportability, to simplify test, integration and maintenance. Both racks are equipped with independent power supply distribution and have their own integrated cooling systems. This paper shows the assembly strategy, reports on the development status and describes the tests performed to verify the system before the integration into the whole instrument.

Resume Introduction En France, 26 equipes regionales ressources de soins palliatifs pediatriques (ERRSPP) ont ete creees entre 2008 et 2012. Nous avons conduit la premiere etude prospective francaise visant a decrire les caracteristiques du recours initial a une ERRSPP et analyser les reponses apportees par cette equipe. Materiel et methodes Toutes les demandes d’interventions formulees a l’ERRSPP entre septembre 2013 et septembre 2014 ont ete recueillies de facon prospective a l’aide d’un questionnaire comportant : les elements demographiques et cliniques du patient (âge, sexe, pathologie), les caracteristiques de la demande (origine du demandeur, specialite du service, motif de la demande) et de la reponse apportee par l’ERRSPP (intervalle diagnostic initial–demande, duree de la prise en charge, deces eventuel, modifications de la demande initiale). Les pathologies ont ete classees dans l’un des 6 groupes de maladies de soins palliatifs pediatriques (SPP), selon les normes edictees par l’Association canadienne de soins palliatifs. Resultats Nous avons recense 67 demandes, concernant 61 patients (37 garcons). L’âge median etait de 49,8 mois [2,3–145]. Les motifs de demande ont ete multiples : reunion ethique pluridisciplinaire (42 %), coordination (34 %), aide a la prise en charge symptomatique (21 %), aide au domicile (19 %), soutien psychologique (15 %), enseignement (9 %) et discussion autour d’un cas (6 %). La demande d’une reunion ethique a ete predominante pour la periode neonatale (61 %) alors que la coordination de la prise en charge l’a ete pour les enfants et adolescents (78 % au-dela de 4 ans). Le delai median « diagnostic–demande » rapporte aux groupes de maladies a ete court pour le groupe 5 (neonatalogie : 0,36 mois) et long pour le groupe 4 (maladies irreversibles et non evolutives : 54,6 mois) (p Conclusion Cette etude montre l’heterogeneite du premier recours a une equipe de SPP, validant ainsi les differentes missions devolues aux ERRSPP et la necessite d’une equipe pluridisciplinaire. La reponse de l’ERRSPP a ete elargie dans la moitie des cas, demontrant une modulation des besoins dans le temps.

Resume Les tumeurs rhabdoides (TRh) composent une famille de tumeurs heterogenes, d’une grande agressivite, survenant chez le jeune enfant. Leur regroupement au sein d’une entite unique est recent, consecutif a la decouverte dans les cellules tumorales d’une inactivation bi-allelique du gene suppresseur de tumeurs hSNF5/INI1. L’inactivation bi-allelique de hSNF5/INI1 retrouvee au niveau constitutionnel dans un tiers des cas permet d’identifier un syndrome de predisposition aux TRh. Nous rapportons trois observations de TRh extra-renales chez des nourrissons âges de 3 a 6 mois, soulignant le caractere trompeur de la presentation clinique, l’agressivite de la maladie, et dont nous detaillons la strategie de prise en charge genetique.

Resume Les formes neurologiques de syndromes de la traversee thoraco-brachiale (STTB) associees a un deficit moteur objectif sont exceptionnelles et rarement individualisees dans la litterature. L’objectif de ce travail etait de preciser leur tableau clinique, les causes anatomiques, les resultats du traitement chirurgical et les facteurs pronostiques. Methodes Parmi les cas de STTB neurologiques, pris en charge entre 1989 et 2011, 30 cas presentant une amyotrophie ont ete diagnostiques chez 28 patients (22 femmes et six hommes) dont l’âge moyen etait de 44 ans (de 16 a 70 ans). Resultats Le deficit moteur predominait sur les muscles thenariens lateraux et l’atteinte etait bilaterale dans deux cas. Vingt-quatre cas (22 patients) ont ete operes. Vingt-trois cas ont pu etre evalues avec un delai moyen de 39 mois (de quatre a 149). Apres le traitement chirurgical, quatre cas (17 %) etaient gueris, neuf cas (39 %) tres ameliores et dix cas (44 %) legerement ameliores. Conclusions Les STTB neurologiques deficitaires realisent une entite qu’il faut differencier des formes douloureuses. Le deficit moteur est toujours d’installation progressive et la douleur, quand elle est presente, reste toujours au second plan. Le pronostic est essentiellement lie au delai d’evolution.

An accurate treatment of first episodes in schizophrenia and bipolar disorders has a significant impact on compliance and prognosis. However, existing therapeutic guidelines may be poorly respected and may concern only typical clinical situations. Medical attitudes in clinical practice have been collected and structured on the basis of small interactive meetings (Focus Group [FG]), and a synthesis of practical attitudes has been compared with updated guidelines. The FG method applied to treatment initiation in schizophrenia and bipolar disorder is seen as complementary to evidence-based guidelines. It reveals that, in a reflexive manner, clinical attitudes are often more diverse and frequently consider first treatments after global evaluation, taking more into account external factors such as clinicians' experience, patient's history and willingness, clinical setting, and environment. A symptomatic approach is sometimes preferred, and a better alliance is always considered as a main objective. The FG method could be a supplementary support to continuous medical education.

Background Children with HIV will be on antiretroviral therapy (ART) longer than adults, and therefore the durability of first-line ART and timing of switch to second-line are key questions. We assess the long-term outcome of protease inhibitor and non-nucleoside reverse transcriptase inhibitor (NNRTI) first-line ART and viral load switch criteria in children. Methods In a randomised open-label factorial trial, we compared effectiveness of two nucleoside reverse transcriptase inhibitors (NRTIs) plus a protease inhibitor versus two NRTIs plus an NNRTI and of switch to second-line ART at a viral load of 1000 copies per mL versus 30,000 copies per mL in previously untreated children infected with HIV from Europe and North and South America. Random assignment was by computer-generated sequentially numbered lists stratified by age, region, and by exposure to perinatal ART. Primary outcome was change in viral load between baseline and 4 years. Analysis was by intention to treat, which we defined as all patients that started treatment. This study is registered with ISRCTN, number ISRCTN73318385. Findings Between Sept 25, 2002, and Sept 7, 2005, 266 children (median age 6.5 years; IQR 2.8-12.9) were randomly assigned treatment regimens: 66 to receive protease inhibitor and switch to second-line at 1000 copies per mL (PI-low), 65 protease inhibitor and switch at 30,000 copies per mL (PI-higher), 68 NNRTI and switch at 1000 copies per mL (NNRTI-low), and 67 NNRTI and switch at 30,000 copies per mL (NNRTI-higher). Median follow-up was 5.0 years (IQR 4.2-6.0) and 188 (71%) children were on first-line ART at trial end. At 4 years, mean reductions in viral load were -3.16 log(10) copies per mL for protease inhibitors versus -3.31 log(10) copies per mL for NNRTIs (difference -0.15 log(10) copies per mL, 95% CI -0.41 to 0.11; p=0.26), and -3.26 log(10) copies per mL for switching at the low versus -3.20 log(10) copies per mL for switching at the higher threshold (difference 0.06 log(10) copies per mL, 95% CI -0.20 to 0.32; p=0.56). Protease inhibitor resistance was uncommon and there was no increase in NRTI resistance in the PI-higher compared with the PI-low group. NNRTI resistance was selected early, and about 10% more children accumulated NRTI mutations in the NNRTI-higher than the NNRTI-low group. Nine children had new CDC stage-C events and 60 had grade 3/4 adverse events; both were balanced across randomised groups. Interpretation Good long-term outcomes were achieved with all treatments strategies. Delayed switching of protease-inhibitor-based ART might be reasonable where future drug options are limited, because the risk of selecting for NRTI and protease-inhibitor resistance is low. Funding Paediatric European Network for Treatment of AIDS (PENTA) and Pediatric AIDS Clinical Trials Group (PACTG/IMPAACT).

Resume Objectifs Les donnees epidemiologiques concernant les tumeurs de la main et du poignet restent peu nombreuses et parfois discordantes. La collaboration au sein du service des chirurgiens de la main et des tumeurs de l’appareil locomoteur a permis d’etudier l’epidemiologie de ces lesions. Methodes Sur la periode de janvier 1980 a septembre 2008, a l’aide du codage diagnostique, de l’etude des comptes-rendus de consultation et du registre du service d’anatomopathologie, une etude retrospective sur le diagnostic de toutes les tumeurs de la main et du poignet operees dans le service a ete realisee. Resultats La moyenne d’âge de la population etait de 48 ans (de 16 a 94 ans). L’effectif regroupait 623 tumeurs traitees chez 620 patients (372 femmes et 248 hommes). Les tumeurs concernaient 525 fois les tissus mous (84,1 %), 70 fois le tissu osseux (11,4 %) et 28 fois le tissu cutane (4,5 %). La prevalence des tumeurs malignes etait de 1,3 %. Conclusions Cette serie correspond au troisieme effectif de la litterature. Les donnees retrouvees sont tres proches des autres travaux publies. La prevalence des tumeurs osseuses (TO) (11,4 %) est plus elevee que dans les autres etudes (de 1,6 a 6,6 %) et celle des tumeurs cutanees plus faible. A l’exception des tumeurs cutanees, dont le mode de recrutement est plus disperse, cette serie donne un bon reflet de la prevalence des differentes tumeurs de la main et du poignet.

This article describes a five-part methodology to identify dust explosion scenarios: (1) a preliminary combustible dust screening, (2) understanding the product and its particle size distribution, (3) gathering and understanding the relevant flammability data, (4) understanding the process steps in a given unit operation that can create dust clouds, and (5) identifying potential ignition sources with the unit operations. By identifying all the potential hazard scenarios, the designer will be better prepared to protect the people and the property involved in processing combustible dusts by incorporating the above safety analysis in the design and specification of the equipment.

This paper analyzes the effects of dollarization, where a country (C) uses money produced by another country (M). We derive a general formula to determine the “optimal” rate of inflation, which maximizes M's welfare but does not take into account any loss to C. We show how this inflation varies with relative income size and output growth. Estimates of the “optimal” inflation rate are made for some countries. We also analyze a contract, under which M shares a fraction of total potential seigniorage with C to induce M to inflate at a rate which leads to no seigniorage accruing to M from C. Finally, we provide estimates of the present discounted value of the seigniorage accruing from M to C. Despite this loss, we estimate the net welfare gain to C's household when dollarization results in a lower rate of inflation.

In France, 26 regional pediatric palliative care teams (ERRSPP) were created between 2008 and 2012. We conducted the first prospective French study to describe the main specifications of the initial contact with an ERRSPP and to analyze the responses given.All the requests for interventions on the part of the ERRSPP were collected between September 2013 and September 2014. We prospectively completed a questionnaire on the patient's clinical and demographic data (age, sex, disease), details regarding the request (type of applicant, unit's specialty, request pattern), and the answers provided (interval between diagnosis and request, duration of care by ERRSPP, death, changes compared to the initial request). The diseases were classified within one of the six groups of palliative pediatric care diseases, based on the standards of the Canadian palliative care association.We gathered 67 requests, 61 relating to patients. The median age at the request was 49.8 months (range: 2.3-145). The original pattern was multiple: multidisciplinary decision-making (42 %), coordination of care (34 %), symptom management (21 %), logistic support for home care (19 %), education (9 %) and case discussion (6 %). Requests concerning multidisciplinary decision-making were predominant within the neonatal period (61 %); coordination of care was significant for children and adolescents (78 % after 4 years of age). The study of the median time from diagnosis to request compared to the groups of diseases revealed a short time in group 5 (neonatology: 0.36 months) and a long time in group 4 (irreversible and nonscalable diseases: 54.6 months) (P0.001). At the end of the study, the follow-up of 50.8 % of the patients by ERRSPP was still going on (median duration of care by the ERRSPP of Languedoc-Roussillon region [ERRSPP-LR], 3.4 months [range: 0.2-5.5]). No request was formulated by a general practitioner.This study shows the heterogeneity of the initial contact made with an ERRSPP, confirming its different assignments and the need for a multidisciplinary team. The ERRSPP's answer was expanded in half of the cases, attesting to the changing needs over time.

The use of an acetylcholinesterase inhibition assay for the detection of dichlorvos in durum wheat samples by a simplified extraction procedure is reported. After an incubation step, the residual activity was determined with an amperometric biosensor using a portable potentiostat. The use of electric eel and recombinant acetylcholinesterase was compared. The effect of the matrix extract was evaluated by using various sample:solvent ratios, 1:2.5, 1:5, 1:10, and 1:20. The optimal extraction ratio, considering the electrochemical interferences and the effect on enzyme activity and bioavailability of the pesticide, was 1:10. Calibrations were performed in buffer and durum wheat extract. The calculated detection limits in buffer solution were 10 ng/ ml and 0.045 ng/ml for electric eel and recombinant acetylcholinesterase, respectively, whereas operating in the matrix extract they increased up to 45 ng/ml and 0.07 ng/ml, corresponding to 0.45 mg/kg (extraction ratio 1:10) and 0.07 mg/kg in samples. These characteristics allowed the detection of contaminated samples at the maximum residue limit, which is 2 mg/kg and well below. Fortified samples of durum wheat were obtained with both dichlorvos and the commercial product Didivane, which contains dichlorvos as an active molecule. At all the tested levels, the occurrence of contaminant was detected with an average recovery of 75%. The total assay time, including the extraction step, was 30 min. Because several extractions as well as most of the assay steps can be run simultaneously, the throughput for one operator is 12 determinations per hour.

National audience; Introduction et but de l’étudeLes inégalités sociales de santé ont une composante nutritionnelle avérée à laquelle pourrait contribuer le coût plus élevé d’une alimentation équilibrée. Cette association entre coût et qualité de l’alimentation n’a jusqu’ici été étudiée qu’à partir de prix alimentaires standards et non à partir de dépenses réelles. Selon la théorie de la déviance positive, l’étude des achats alimentaires réels devrait permettre d’identifier des foyers de faible statut socio-économique (SES) dont les achats sont de meilleure qualité nutritionnelle. Le but l’étude était d’étudier la relation entre les dépenses alimentaires réelles et la qualité nutritionnelle de l’alimentation au sein d’une population de faible SES, et identifier des « déviants positifs » afin de dégager les caractéristiques de leurs achats alimentaires.Matériel et méthodesDes personnes habitant dans des quartiers défavorisés à Marseille (n = 91) ont été recrutées pour participer à l’intervention d’éducation nutritionnelle Opticourses (opticourses.fr). Elles ont collecté les tickets de caisse des achats alimentaires de leur foyer pendant un mois. Les dépenses alimentaires effectives par jour et par personne (e/j.p.) ainsi que le coût de l’énergie (e/2 000 kcal d’achats) ont ensuite été calculés et corrélés à trois indicateurs de qualité nutritionnelle : le « Mean Adequacy Ratio » (MAR), le « Mean Excess Ratio » (MER) et la densité énergétique (DE). Le MAR et le MER ont été utilisés pour identifier 21 foyers « déviants positifs », c’est-à-dire les foyers dont les achats présentaient une meilleure qualité nutritionnelle (MAR > médiane et MER < médiane).Résultats et Analyse statistiqueUn coût d’énergie plus élevé était associé à un plus fort MAR et à une plus faible DE. Les achats alimentaires des « déviants positifs » avaient une qualité nutritionnelle significativement plus élevée que ceux des autres foyers (+13 % de MAR, −90 % de MER, −22 % de DE), pour un coût similaire (3,34 e/j.p. et 3,17 e/2 000 kcal). On observait dans leurs achats, plus de poisson, plus d’œufs, plus de féculents et plus de fruits et légumes, mais moins d’aliments gras, salés et sucrés, en particulier moins de sucreries, de boissons sucrées et de snacks salés, et enfin moins d’aliments consommés sur le pouce comme des sandwichs ou des pizzas.ConclusionNos résultats confirment que l’association positive entre le coût de l’alimentation et sa qualité nutritionnelle persiste lorsque le coût est mesuré par les dépenses réelles, et non pas estimé sur la base de prix alimentaires standards comme dans toutes les études précédentes. De plus, nos résultats indiquent que, en dépit de la relation globalement positive entre coût et qualité nutritionnelle, certains foyers de faible SES font des achats alimentaires plus sains sans dépenser plus. Les comportements d’achat de ces « déviants positifs » confortent la pertinence de mener des actions qui, comme Opticourses, visent à rendre visibles, disponibles et attractifs les aliments de bon rapport qualité nutritionnelle/prix pour faciliter l’atteinte de l’équilibre nutritionnel avec un petit budget.

Elderly people commonly suffer from dyspnoea, which may stem from expiratory flow limitation (EFL). The relationship between EFL, as assessed by the negative expiratory pressure method and spirometric indices, was investigated in an elderly French population. Subjects, aged 66-88 yrs, filled in socio-demographic and standardised questionnaires, which dealt with: medical history, smoking status and respiratory symptoms. EFL measurements and forced expiratory manoeuvres were performed. Validated measurements were obtained in 750 out of 1,318 subjects: 47% were EFL+ (EFL >0), with a higher prevalence in females than in males. EFL and forced expiratory volume in one second (FEV1) were correlated with age. A total of 116, from the 750 subjects, with no medical history and no symptoms, served as a healthy group. The prevalence of EFL+ subjects increased with the grade of dyspnoea and was highest in respiratory and cardiac patients when compared with the healthy subjects. EFL did not correlate with FEV1/forced vital capacity (FVC), the usual index of obstruction. Some elderly subjects (15%) with dyspnoea but with no medical history, mainly females with small FVC and normal FEV1/FVC, had a greater EFL than the healthy subjects. In elderly people, expiratory flow limitation measurements, along with the usual forced expiratory volume in one second/ forced vital capacity ratio, may be of value for the interpretation of dyspnoea.

Background Because of its unique mechanism of action and safety profile, imiquimod, a topical immune response modifier, is used for many benign and malignant dermatologic conditions. Adverse effects are typically limited to treatment site erythema and erosion. Objective To describe a newly recognized adverse effect of imiquimod. Methods A 79-year-old woman being treated with imiquimod 5 days per week for a nodular basal cell developed a verrucous plaque over the treatment area after 7 weeks of therapy. Results Scouting biopsies demonstrated multiple comedones and ruptured epidermoid cysts. There was no evidence of residual basal cell carcinoma. Conclusions Imiquimod is a new and novel treatment option for cutaneous malignancies. We report its successful use in the treatment of a nodular basal cell carcinoma. The multiple comedones and ruptured epidermoid cysts are newly reported adverse effects of imiquimod therapy.

Background: In the last decades in industrialized countries, the increase of life expectancy has resulted in an increase in the population of the elderly. However, little is known about the prevalence of allergies in the elderly population. The aim of the study was to investigate the specific relationship of serum IgE and Phadiatop, with asthma, rhinitis, and smoking in a sample of an elderly population. Methods: Subjects from the Paquid cohort living in Gironde Department (age 65 years and over) in France were followed up since 1988 (PAQUID cohort). Results: Among the randomized sample of 352 subjects, there were 158 men (45%) and 194 women (55%). The lowest levels of IgE were in subjects with chronic sputum; and in normal subjects (47.1 ± 56.4 vs 56.2 ± 73.9, NS). Multiple linear regression was performed with log10 IgE values as a dependent variable and age, Phadiatop test, smoking, and respiratory symptoms independently in men and in women. In men, a significant relationship was observed between IgE values and Phadiatop test (P 65 years of age. There is an association between smoking and IgE level independent of allergic reactivity to common allergens in the elderly.

Extensive observations and experiments suggest that collisions with plate glass result in more avian mortalities than any other human-associated factor. We tested the effects of window angling and the distance of bird feeders from windows on bird-glass collisions. Strike frequency differed among windows oriented vertically (control) and those angled 20 and 40 degrees from vertical; as the angle of orientation increased, strikes and fatalities decreased. Strike frequency and fatalities at windows also increased as the distance between bird feeders and the glass surface increased. No fatalities were recorded when feeders were located within 1 m of a window, but a marked increase in mortality occurred when feeders were placed 5 and 10 m from the glass. Most glass-collision victims may go unnoticed, hidden by vegetation where they remain out of view or are removed by scavengers. We found that scavengers frequently removed baits from beneath windows at six buildings, but no baits were taken from a sit...

This paper describes the Electrical Ground Support Equipment (EGSE) developed for automatic tests of DREAMS (Dust characterization, Risk assessment, and Environment Analyser on the Martian Surface), that is the only surface payload package to be accommodated on the Entry, Descendent and landing Module (EDM) of the ExoMars 2016 mission to Mars, developed by the European Space Agency (ESA) in collaboration with the Russian Federal Space Agency (Roscosmos).

Rhabdoid tumors are a heterogeneous family of aggressive tumors affecting young children. Their grouping within a single entity is recent, following the discovery of a bi-allelic inactivation of the hSNF5/INI1 tumor suppressor gene in tumoral cells. This bi-allelic inactivation of the hSNF5/INI1 gene found at the constitutional level in up to one-third of cases has led to the identification of a predisposal syndrome to rhabdoid tumors. Herein we report extrarenal rhabdoid tumors observed in three infants between 3 and 6 months of age, underlining the misleading feature of the clinical presentation and the aggressiveness of the disease. Finally, we also report the genetic patient care management strategy.

The potential role of unrelated donor cord blood transplantation (UD-CBT) in adults remains unclear. This study reports the results of UD-CBT in 22 adults with hematologic malignancies following conditioning with thiotepa, busulfan, cyclophosphamide, and antithymocyte globulin in 21, with thiotepa, fludarabine, and antithymocyte globulin in 1, and graft-versus-host disease (GVHD) prophylaxis with cyclosporine and prednisone. Median age was 29 years (range, 18-46 years), and median weight was 69.5 kg (range, 41-85 kg). HLA match was 6 of 6 in 1 case, 5 of 6 in 13 cases, and 4 of 6 in 8 cases. Median number of nucleated cells infused was 1.71 × 107/kg (range, 1.01 × 107/kg to 4.96 × 107/kg). All 20 patients surviving more than 30 days had myeloid engraftment, and only 1, who received the lowest cell dose, developed secondary graft failure. Median time to reach an absolute neutrophil count of at least 0.5 × 109/L was 22 days (range, 13-52 days). Median time to platelets numbered at least 20 × 109/L was 69 days (range, 49-153 days). Seven patients (32%) developed acute GVHD above grade II, and 9 of 10 patients at risk developed chronic GVHD, which became extensive in 4 patients. Twelve patients remained alive and disease-free 3 to 45 months after transplantation. Disease-free survival (DFS) at 1 year was 53%. Age strongly influenced DFS (P = .01). For patients aged 30 years or younger, the DFS at 1 year was 73%. These preliminary results suggest that UD-CBT should be considered a reasonable alternative in young adults with hematologic malignancy and no appropriate bone marrow donor.

Introduction La vasculopathie cutanee collagenique (VCC) est une vasculopathie primitive idiopathique a l’origine de telangiectasies acquises sans atteinte systemique et avec des caracteristiques histologiques particulieres. Nous rapportons une serie de 7 cas. Materiel et methodes Etude retrospective des caracteristiques cliniques, dermatoscopiques et histologiques de patients avec une VCC. Observations Sept patients (6 femmes, 1 homme) d’âge moyen 53 ans (12–80) ont ete inclus. Resultats Les telangiectasies evoluaient entre 1 et 10 ans et etaient non infiltrees et non palpables, le plus souvent asymptomatiques (hypersensibilite dans 1 cas), de distribution symetrique sur les pieds (2), les jambes et les cuisses (5), les bras (1), l’abdomen (2), les seins (1) et des vergetures (1). La face et les muqueuses etaient indemnes. La dermatoscopie montrait des vaisseaux capillaires rouges, dilates, serpigineux, ramifies, parfois en grain de chapelets. L’histologie etait caracteristique avec des vaisseaux capillaires du derme superficiel dilates avec un epaississement de la paroi vasculaire et un materiel amorphe, hyalin, eosinophile, positif au PAS. Des depots de collagene IV etaient cherches et trouves dans un cas. Il n’y avait pas de vasculite, de thrombose, d’hemorragies, d’amyloidose ou d’important infiltrat inflammatoire. La prise en charge etait difficile. Les flavonoides etaient inefficaces dans un cas. La brimonidine 0,33 % (Mirvaso®) nous a semble donner des signes d’amelioration chez la plus jeune patiente mais le cout limitait son utilisation. Plusieurs patients refusaient un traitement par laser pour des raisons financieres. Discussion La VCC est une vasculopathie benigne d’individualisation recente (2000) et de physiopathogenie mal connue (microthrombose occlusive sur agression endotheliale ?). Les telangiectasies des membres inferieurs pouvant s’etendre progressivement a l’abdomen, au tronc et aux extremites superieures dans les mois et les annees suivantes. Elle touche les sujets entre 40 et 80 ans. Mais des formes juveniles ont ete rapportees. Nous rapportons le plus jeune cas diagnostique a ce jour (diagnostic a 12 ans mais debut des symptomes a l’âge de 6). L’histologie est caracteristique avec une dilatation des capillaires et un depot de materiel hyalin amorphe de collagene IV. La dermatoscopie est assez stereotypee, avec quelques differences selon l’âge des lesions. Le traitement n’est pas codifie. La VCC ne mettant pas en jeu le pronostic vital, le traitement est propose si retentissement esthetique. Un traitement par laser a colorant pulse 585 nm ou par IPL pourrait avoir une efficacite. La brimonidine 0,33 % nous a semble une alternative a explorer. Conclusion Le dermatologue doit savoir reconnaitre cette nouvelle entite car elle est probablement sous-diagnostiquee. Les telangiectasies generalisees essentielles sont le diagnostic differentiel principal.

We describe our experience in the management of 11 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids. Nine patients (82%) achieved complete remission (CR) after a median of 6 rounds of PE (range 2–22). There were 3 early relapses managed in the same way as the initial episode. One patient relapsed 23 months after diagnosis achieving CR with standard therapy; another patient suffered several relapses, and splenectomy was performed after the last one. Three patients died, 2 of them with resistant disease 9 and 38 days after diagnosis, and the remaining one died due to AIDS-related complications while he was in CR. Eight patients are alive in CR with a median follow-up of 38 months (range 8–74). The combination of PE and steroids is a well-tolerated and effective treatment of TTP, but improvements in therapy are needed to manage refractory patients.

A new vibrating-tube densimeter has been constructed for measuring the relative densities of fluid systems in the elevated temperature and pressure ranges 323≤ T /K≤533 and 0.1≤ p /MPa≤40. The instrument utilizes a light beam in the collection of a signal and dispenses with the usual pick-up and drive wire combinations required in other instruments. The densimeter has been tested by measuring relative densities for aqueous sodium chloride solutions at temperatures from T =398 K to T =473 K and pressures from p =10 MPa to p =30 MPa. Calculated densities have been compared to those previously published in the literature in order to assess the precision of our new instrument. Also, relative densities for glycine in water have been measured at temperatures from T =398 K to T =473 K and pressures from p =10 MPa to p =30 MPa over an extended concentration range. Calculated standard state volumes are compared to those which may be calculated from previously published revised Helgeson, Kirkham, and Flowers equations of state.

Background: Gastric emptying scintiscans are currently used to select reflux patients for added pyloroplasty at the time of fundoplication. The accuracy of this scan selection approach has been assumed. If preoperative scintiscans do not reliably predict postfundoplication gastric emptying, however, the decision to add pyloroplasty to the fundoplication operation may be inappropriate and even harmful. Methods: The authors studied 27 children prospectively before and after gastric fundoplication. Gastric emptying at 60 minutes was measured by double isotopic labeling of liquid ( 111 In) and solid ( 99m Tc) phases of a test meal specifically designed for label fixation. The authors' question involved the accuracy of preoperative gastric scintiscans in predicting postfundoplication delay of gastric emptying (DGE). An evaluation of pyloroplasty as an effective treatment for DGE was not part of the study design. Pyloroplasty was performed as a secondary operation in three of the study children, however, because they persisted with unrelieved symptoms of retching, fullness, and abdominal discomfort. Scintiscan-documented postfundoplication delay in gastric emptying was present in all three patients at 18, 58 and 12 weeks, respectively. Additional scintiscans were performed in these patients after pyloroplasty. Results: Gastric emptying of solids at 60 minutes did not show a significant change after a gastric fundoplication operation, although the trend was in the direction of a decrease (paired t test, P = . 13). Liquid emptying at 60 minutes, however, was significantly increased (paired t test, P = .01). The variation in values between patients was wide, and the correlation between pre- and postoperative study results in the same patient was poor (r 2 = 0.337 for solids and r 2 = 0.116 for liquids). Most unexpectedly, scintiscans after postfundoplication pyloroplasty in the three patients with persistent symptoms showed no improvement in delayed gastric emptying on repeat scintiscan 42 to 117 weeks later. Conclusions: The data suggest that preoperative scintiscan evidence for postfundoplication DGE is probably accurate for solid emptying but not for liquids, at least as measured by the double isotope methodology of our study. Preoperative scintiscans that use a liquid phase label only may be highly misleading for the prediction of postfundoplication DGE. Furthermore, pyloroplasty may not be useful as treatment even when postfundoplication delay in gastric emptying can be accurately anticipated or confirmed. A fundamental motility disorder of the gastric body seems to be more important than muscular resistance at the gastric outlet as a cause for postfundoplication DGE, and the most effective treatment approach remains unclear.

Mitochondrial activity requires the expression of nuclear genes, whose products are part of multiproteic complexes leading to ATP production and delivery. We recently characterized a growth-activated mRNA encoding the human mitochondrial ribosomal MRPL12 protein, which is thought to act as a translational regulator of mitochondrial mRNAs. We show here that MRPL12 mRNA expression is enhanced in growth-stimulated cells as a result of transcriptional activation, a feature lost in transformed cell lines. MRPL12 mRNA is highly expressed in the colon, in which a reduction in mitochondrial activity was shown to be associated with tumor formation. The human MRPL12 protein is encoded by a unique gene located on chromosome 17 (q25–qter). As no predisposition to colon cancer linked to this chromosomal region was hitherto reported, the MRPL12 gene might be involved in the process of differentiation of colonic epithelial cells.

Relative densities, [Formula: see text] and heat capacity ratios, [Formula: see text] have been measured for aqueous solutions of glycine, glycylglycine, and glycylglycylglycine in aqueous urea solutions containing 1–13 mol kg−1 urea. These data have been used to calculate apparent molar volumes, [Formula: see text] and apparent molar heat capacities, [Formula: see text] which in turn have been used to obtain standard state volumes, [Formula: see text] and standard state heat capacities, [Formula: see text].Volumes and heat capacities of transfer from water to urea have been calculated from our reported standard state data. Heat capacities of transfer of the investigated systems have been modeled using a procedure developed from the semiempirical calculation procedures proposed by Helgeson, Kirkham, and Flowers. Keywords: amino acids, peptides, calorimetry, transfer thermodynamics, HKF theory.

A reagentless acetaldehyde sensor is designed based on the co-entrapment of aldehyde dehydrogenase (AIDH), NADH oxidase and high molecular weight NADs (NAD-dextran and NAD-PEG) in a polyvinyl alcohol) bearing stryrylpyridinium groups (PVA-SbQ) matrix. The amperometric detection is based on hydrogen peroxide oxidation by applying a potential difference of 550 mV between two platinum electrodes, equivalent to ca. 600 mV vs. SCE. The sensitivity is close to 2 mA 1 mol−1, the linear range extends from 0.5 μM to 330 μM acetaldehyde and the response time is lower than 3 minutes. More than 80 reproducible measurements can be performed without cofactor addition, indicating a good operational stability. Acetaldehyde concentration in alcoholic beverages is determined and validation of the results is made using a classical spectrophotometric method.

Background This study reports the incidence of Wr(a) antigen and anti-Wr(a) in Valencia, Spain. Study design and methods The incidence of the Wr(a) antigen in 110,000 healthy blood donors was estimated. Likewise, the incidence of anti-Wr(a) was analyzed in a population consisting of 730 healthy blood donors, 356 pregnant women, and 581 patients who received transfusions from the area of Valencia, Spain. Results The incidence of Wr(a) antigen was 1 in 785. Overall, anti-Wr(a) was found in 59 samples: 20 healthy blood donors (1/37), 18 pregnant women (1/20), and 21 patients who received transfusions (1/28). The most frequent immunoglobulin class of anti-Wr(a) in healthy blood donors was immunoglobulin M, either alone (8 cases) or plus immunoglobulin G (IgG; 8 cases); the IgG1 and IgG3 were the IgG subclasses most frequently detected in pregnant women (12 cases) and in patients who received transfusions (12 cases). Only 51 percent of the anti-Wr(a) appeared to have the potential to be clinically significant. Conclusion These data show that the incidence of Wr(a) antigen and anti-Wr(a) among the population from Valencia is similar to that reported in other European areas and suggest that the development of anti-Wr(a) is facilitated by the presence of a hyperactive immune system. The clinical relevance of anti-Wr(a) is limited, however.

Unanticipated death from enterocolitis occurred in five children 3 weeks to 20 months after uncomplicated reconstruction for Hirschsprung's disease. In each case the presenting symptoms of enterocolitis were mild and were misinterpreted by examining physicians. Within 2 to 12 days of onset of symptoms, unexpected death occurred. Although fatal enterocolitis is a well-known complication of Hirschsprung's disease, emphasis is usually placed on preoperative enterocolitis. Fatal postoperative enterocolitis is not a new entity associated with Hirschsprung's disease, but physician awareness of this possibility is obviously deficient. We strongly recommend extensive parent education and better postoperative communication between the surgeon and the referring physician.

This study is a retrospective review of all children treated for Hirschsprung's disease over the past 22 years at a single pediatric institution. During this time 177 patients had definitive surgical reconstruction. Five children died of causes unrelated to Hirschsprung's disease, and five children died from enterocolitis after an uneventful postoperative course. Clinical follow-up information was obtained from 135 (78%). Demographic data includes the following: sex ratio 74% male, 26% female; current mean age 9.9 years; mean length of follow-up 7.9 years (range, 3 months to 21.5 years). Mean age at surgical reconstruction was 1.6 years. Definitive surgical procedures included endorectal pull-through (Soave), 21%; modified Duhamel, 67%; extended side-to-side ileocolic anastomosis, 8%; rectal myomectomy, 4%. Transition zone was within rectum or rectosigmoid region in 86%. Overall, 32% (43/135) report difficulty with fecal soiling, and 12.6% (17/135) identify this as a severe problem. These numbers include patients with trisomy 21 and total colonic aganglionosis. Severe fecal soiling was reported in 7.1% (2/28) after an endorectal pull-through, and in 12.1% (11/91) after the modified Duhamel. The difference in incidence of soiling after these two procedures is not statistically significant. However, 40% (4/10) of the patients after the long side-to-side anastomosis for total colonic aganglionosis report severe problems with fecal soiling (P = .03). Surgical reconstruction for Hirschsprung's disease provides near-normal gastrointestinal function for the majority of children, but long-term follow-up shows significant residual problems with soiling in 12.6% of the patients. This is consistent with reported experience worldwide.

Densities and heat capacities have been measured for aqueous solutions of L-asparagine, L-glutamine, glycylglycine, glycyl-L-valine, glycyl-L-asparagine, and glycyl-DL-leucine at 288.15, 298.15, 313.15, and 328.15 K. These data have been used to calculate apparent molar volumes, V2,ø, and apparent molar heat capacities, Cp,2,ø, which in turn have been used to obtain standard state volumes, [Formula: see text] and heat capacities, [Formula: see text] The semi-empirical modelling procedures of Helgeson, Kirkham, and Flowers have been used to subdivide the calculated standard state volume and heat capacity data into solvation and nonsolvation contributions. The nonsolvation components of the standard state properties are used in group additivity analyses. These analyses yield structural contributions to standard state volumes and heat capacities for the CH(NH2)CO2H, CH2, OH, COOH, CH, CONH2, and CONH groups. The temperature dependences of these contributions are discussed. Some comments are reported concerning the practicality of using the thermodynamic properties of aqueous amino acid and peptide systems as the basis for modelling standard state thermodynamic properties of aqueous protein systems. Keywords: heat capacities, densities, volumes, amino acids, peptides, group additivity.

Onychomycosis is difficult to treat. Systemic therapy with the potent oral azoles may be restricted by their potential side-effects; an attractive alternative is topical application of an antifungal directly to the nail. Several such formulations have been developed, including creams and lotions which are largely ineffective due to poor drug penetration into and through the nail structure. Nail keratin is thick and compact; its permeability is low. Transungual drug diffusion depends on the characteristics of the nail (especially degree of hydration) and the properties of the chemical (molecular weight and size, and lipophilic/hydrophilic profile). A nail lacquer containing 5% amorolfine was recently introduced; the volatile vehicle evaporates, leaving an occlusive film on the surface of the nail. The film acts as a drug depot, while at the same time increasing the hydration of the nail and the thermodynamic activity of the drug, thereby enhancing diffusion, particularly of hydrophilic compounds. Amorolfine has been shown to penetrate human nail from the film at clinically effective concentrations. In addition, the effect is long lasting: a single application of lacquer provides protection for 1 week. Release and rate of diffusion can be optimized by selecting the components of the lacquer formulation (solvent, polymer, plasticizer). Transungual drug delivery via nail lacquer is a major addition in the dermatologist's therapeutic arsenal.

Neurologic TOS with amyotrophy is exceptional and the diagnosis is usually delayed compromising the outcome after treatment. The aims of this study were to define clinical features, anatomical causes, surgical results and prognostic factors of this disease.All of the TOS with objective neurologic deficit treated in our unit between 1989 and 2011 were assessed retrospectively. Thirty cases were identified in 28 patients (two bilateral cases). The mean-age was 44 years (16 to 70).Muscular atrophy always predominated in the lateral thenar muscles. Seventeen also had sensory symptoms. Twenty-two patients (24 cases) were surgically treated. Twenty-three cases could be assessed with a mean follow-up of 39 months. Recovery was complete in four cases (17%), marked in nine cases (39%) and minimal in ten cases (44%).Neurological TOS with objective manifestations have to be distinguished from other types of neurological TOS. Muscular atrophy in the hand appears insidiously and the pain, which is inconstant, stays always in the background. Duration of symptoms before surgical treatment is the main prognostic factor.

As an increasing number of patients undergo successful solid organ transplantation, immunocompromised patients are encountered more commonly in the private practice office. Thus the evaluation of such patients should take into consideration the possibility of infection. We report the case of a kidney transplant recipient who took standard immunosuppressive therapy and presented with cutaneous findings of secondary syphilis. Skin biopsy and serologic testing confirmed the diagnosis. The patient was treated according to current guidelines from the Centers for Disease Control and Prevention. We present a brief review of the clinical presentation, pathologic findings, diagnostic methods, and treatment options for syphilis.

An accurate treatment of first episodes in schizophrenia and bipolar disorders has a significant impact on compliance and prognosis. However, existing therapeutic guidelines may be poorly respected and may concern only typical clinical situations. Medical attitudes in clinical practice have been collected and structured on the basis of small interactive meetings (Focus Group [FG]), and a synthesis of practical attitudes has been compared with updated guidelines. The FG method applied to treatment initiation in schizophrenia and bipolar disorder is seen as complementary to evidence-based guidelines. It reveals that, in a reflexive manner, clinical attitudes are often more diverse and frequently consider first treatments after global evaluation, taking more into account external factors such as clinicians' experience, patient's history and willingness, clinical setting, and environment. A symptomatic approach is sometimes preferred, and a better alliance is always considered as a main objective. The FG method could be a supplementary support to continuous medical education.

Chloroplasts isolated from spinach were immobilized by entrapment in polyvinyl alcohol bearing styrylpyridinium groups (PVA-SbQ). The best conditions of oxygen production (temperature, pH) were optimized and compared with native chloroplasts. The storage stability in a dry state was good. The results achieved with diuron could allow the use of entrapped chloroplasts to elaborate a method for the detection of pollutants inhibiting the photosynthetic electron flow.

To estimate the relation between resistance to methicillin and virulence in strains of Staph. aureus by estimating the survival at day 30.Case control analysis.Inpatients at a major Cancer Referral Center.21 patients with methicillin-resistant Staph. aureus (MRSA) bactaeremia and 45 patients with methicillin-susceptible Staph. aureus (MSSA) bactaeremia, all treated with vancomycin.Fewer MRSA than MSSA patients were neutropenic (p = 0.04), but more MRSA patients were hospitalized in intensive care units (p = 0.03), had had surgical episodes (p = 0.06). MRSA patients also had more microbiological samples cultured (p = 0.007). The outcome of the bacteriemic episodes in 14 MRSA and 14 MSSA patients matched for these four factors showed that blood cultures from MRSA patients remained positive significantly longer (p = 0.04), but that survival and length of hospital stay were not significantly different.These results do not indicate that methicillin-resistance in Staph. aureus is associated with increased virulence.

Summary. Twenty patients with thrombotic thrombocytopenic purpura (TTP) underwent plasma exchange using either standard fresh-frozen plasma (Group A, na 13) or methylene blue-treated plasma (Group B, na 7). Both groups presented similar characteristics except that bilirubin values were higher in Group A (P , 0·05). The complete remission rate was higher in Group A than B (69% versus 57%). The mean number of procedures was higher in Group B (21 ^ 7 versus 11 ^ 3, P , 0·01) and the mean duration of hospitalization was also longer (37 ^ 12 d versus 22 ^ 11 d; P , 0·01). Our study shows that the use of methylene blue-treated fresh-frozen plasma to treat TTP is associated with a higher number of plasma exchanges and greater transfusion requirements without improving clinical results.

A model is presented to determine the effect on the slope of the LM schedule of full deregulation of bank's interest rates on demand deposits. The opportunity costs of holding a bank deposits is analyzed and explicit consideration is given to the marginal costs of intermediation. Two popular demand functions for money are assumed: the double log and the semi-log functions. It is shown that a strong presumption exists that the slope of the LM schedule is steepened around the fixed full employment real interest rate. Only an unusual increase in the interest elasticity of the demand for money sufficient to offset the impact of other variables could upset this presumption.