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Background: Niemann-Pick disease type C (NP-C) is a rare neurovisceral lysosomal lipid storage disease characterized by progressive neurodegeneration and premature death. While miglustat can stabilize neurological manifestations in later onset forms of NP-C, its efficacy in the early-infantile neurological form has not been demonstrated. In this observational retrospective study, we compared long-term neurodevelopmental outcome and survival between an untreated and a treated group of early infantile NP-C patients. Methods: Data available on all NP-C patients with early infantile neurological onset diagnosed in France between 1990 and 2013 were compiled. Patients with incomplete data or who had died from a systemic perinatal, rapidly fatal form were excluded. Results: Ten patients were included in the treated group (year of birth: 2006–2012), and 16 patients in the untreated group [born 1987–2005 (n = 15), 2012 (n = 1)]. The median age at neurological onset was 9 months (5–18) in the treated group, and 12 months (3–18) in the untreated group (p = 0.22). Miglustat therapy was started at a median age of 24.5 months (9–29) and median duration was 30 months (11–56). Gastrointestinal adverse events were reported in 7/10 patients on miglustat. All patients developed loss of psychomotor acquisitions or additional neurological symptoms despite miglustat therapy. The ages of developmental milestones and neurological involvement did not significantly differ between the two groups. Four patients in the untreated group were lost to follow up. The 22 remaining patients had died by the end of the study and no patient survived beyond the age of 7.4 years. The median survival age was 4.42 years in the untreated group and 5.56 years in the treated group; the Kaplan–Meier survival curves were not significantly different (log-rank test: p = 0.11). Conclusions: Miglustat allowed no significant long-term neurodevelopmental improvement nor significant increase of survival in patients with early infantile NP-C. [ABSTRACT FROM AUTHOR]

The large number of experimentally determined molecular structures has led to the development of a new semiotic system in the life sciences, with increasing use of accurate molecular representations. To determine how this change impacts students' learning, we incorporated image tests into our introductory cell biology course. Groups of students used a single text dealing with signal transduction, which was supplemented with images made in one of three iconographic styles. Typically, we employed realistic renderings, using computer-generated Protein Data Bank (PDB) structures; realistic-schematic renderings, using shapes inspired by PDB structures; or schematic renderings, using simple geometric shapes to represent cellular components. The control group received a list of keywords. When students were asked to draw and describe the process in their own style and to reply to multiple-choice questions, the three iconographic approaches equally improved the overall outcome of the tests (relative to keywords). Students found the three approaches equally useful but, when asked to select a preferred style, they largely favored a realistic-schematic style. When students were asked to annotate "raw" realistic images, both keywords and schematic representations failed to prepare them for this task. We conclude that supplementary images facilitate the comprehension process and despite their visual clutter, realistic representations do not hinder learning in an introductory course. (Contains 7 figures.)

The number of experimentally derived structures of cellular components is rapidly expanding, and this phenomenon is accompanied by the development of a new semiotic system for teaching. The infographic approach is shifting from a schematic toward a more realistic representation of cellular components. By realistic we mean artist-prepared or computer graphic images that closely resemble experimentally derived structures and are characterized by a low level of styling and simplification. This change brings about a new challenge for teachers: designing course instructions that allow students to interpret these images in a meaningful way. To determine how students deal with this change, we designed several image-based, in-course assessments. The images were highly relevant for the cell biology course but did not resemble any of the images in the teaching documents. We asked students to label the cellular components, describe their function, or both. What we learned from these tests is that realistic images, with a higher apparent level of complexity, do not deter students from investigating their meaning. When given a choice, the students do not necessarily choose the most simplified representation, and they were sensitive to functional indications embedded in realistic images. (Contains 8 figures and 5 tables.)

Information and communication technologies (ICTs) are an integral part of our environment, and their uses vary across generations and among individuals. Today's student population is made up of "digital natives" who have grown up under the ubiquitous influence of digital technologies, and for whom the use of ICT is common and whose daily activities are structured around media use. The aim of this study is to examine the impact of ICT use and digital skills on students' academic performance and to explore the digital divide in France. Data were collected through face-to-face questionnaires administered to 1323 students enrolled in three French universities. Principal component analysis, a non-hierarchical k-means clustering approach and multilevel ordered logistic regression were used for data analysis and provide four main findings: first, poor investment in ICT affects students' results; second, the ICT training offered by universities has little impact on students' results; third, student performance improves with the innovative and collaborative use of ICTs; fourth, the acquisition of digital skills increases students' academic performance. The results show that the digital divide still exists, and this raises questions about the effectiveness of education policies in France. They suggest also that organizational change in universities is essential to enable an exploitation of ICT. [ABSTRACT FROM AUTHOR]

• What is already known: The practice of paediatric anaesthesia is highly variable in Europe. The participation of French centres to the APRICOT study allows comparison with other practices in Europe. • What this article adds: The current study describes the epidemiologic and perianaesthetic data of the population of children anaesthetised in the French centres that participated to APRICOT and determine some key points for improving perioperative safety. • Implications for translation: a wider spread of some key elements about safety during perioperative management in children has to be undertaken. Analysing national patients' profile and organisation of human resources are important for improving the perioperative quality of care. The aim of the current study was to achieve these goals using the French data from the APRICOT study. Data from the French centres that participated to the APRICOT study were extracted and analysed. The primary goal of the study was to describe patients' characteristics, procedures and perioperative anaesthetic management in France, and compare them to the results of the European APRICOT trial. Secondary outcomes were the description of major perioperative complications and the determination of human resources organisation possibly associated with these perioperative complications. Overall 3535 procedures collected in 20 facilities (17 teaching hospitals, one community hospital and two private institutions) were analysed. Comparison between the French and European APRICOT cohorts found differences related to the more specialised French centres participating to the study. Overall complications (respiratory complications, haemodynamic instability, cardiac arrest, drug errors, and anaphylactic reactions) were observed in 6.4% [95% CI: 5.6; 6.3] of cases. Multivariate analysis identified the anaesthesiologist's experience of < 15 years and the absence of an anaesthetic nurse as human factors independently associated with an increased risk for perioperative complications. The current study identified some important differences between the French and the whole APRICOT cohort in terms of preoperative evaluation, surgical specialties involved, and monitoring of neuromuscular blockade. It confirms that, in France, the presence of an anaesthetic nurse and an experienced anaesthesiologist prevents anaesthetic complications. [ABSTRACT FROM AUTHOR]

Driven by Information Technologies (IT), universities are making in depth changes to the nature and form of learning processes, which are intended to better prepare students for entry into the labour market. In this paper, we focus on the evolution of students' use of IT in an institution characterized by organizational changes, and we analyse the determinants of students' e-skills using a sample set of French university students. We show that students' involvement in the use of IT increases their e-skills. IT learning by doing and IT learning by using also increase some categories of students' e-skills. In addition, collaborative and cooperative learning are positively associated with students' advanced e-skills. [ABSTRACT FROM AUTHOR]

Neonatal and infant anaesthesia are associated with a high risk of perioperative complications. The aim of the current study was to describe those risks in France using the French data from the NECTARINE study. Data from the French centres that participated to the NECTARINE study were analysed. The primary goal of the study was the description of patients' characteristics, procedures and perioperative management and their comparison with the results of the European NECTARINE study. Secondary outcomes were the description of major perioperative complications and death. Overall, 926 procedures collected in 15 centres (all teaching hospitals) were analysed. Comparison between the French and European NECTARINE cohorts found few differences related to patients' characteristics and procedures. The rate of interventions for critical events (respiratory, haemodynamic, and metabolic) was similar between the two cohorts. Near-infrared spectroscopy monitoring was used in 12% of procedures. Nearly none of the thresholds for these interventions met the published standards. By day 30, complications (respiratory, haemodynamic, metabolic, renal, and liver failure) and death were observed in 14.4% [95% CI 11.6–16.4]% and 1.8% [95% CI 1.1–2.9] of cases, respectively. Although the health status of the patients in the French cohort was less severe, procedures, management and postoperative complications and mortality rates were similar to the European cohort. However, thresholds for interventions were often inadequate in both cohorts. Efforts should be undertaken to improve the knowledge and use of new monitoring devices in this population. [ABSTRACT FROM AUTHOR]

This survey aims to describe current practice in ambulatory care among pediatric anesthesiologists in France. Members of the French-speaking Pediatric Anesthesiologists Association (ADARPEF) were sent a questionnaire examining the proportion of pediatric ambulatory anesthesia practiced by each responder, the level of adherence to pediatric ambulatory Anesthesia guidelines, and responder consensus in decision making when faced with common case scenarios in pediatric ambulatory anesthesia. For the latter, consensus was defined as a > 80% opinion. One hundred and forty-five pediatric anesthesiologists replied (43%). Ambulatory anesthesia appears underused in France. Recent French pediatric ambulatory anesthesia guidelines are being applied. Postoperative pain is poorly managed. The choice of scheduling children for ambulatory anesthesia appears to be more heavily influenced by practitioners' subjective evaluation than evidence from the literature. A better commitment for ambulatory care must be found among anesthesiologists. Further studies are required to improve coherence, safety, and efficiency of children selection for ambulatory anesthesia. [ABSTRACT FROM AUTHOR]

Background: Renal transplantation is the best available therapeutic option for end‐stage renal failure in both children and adults. However, little is known about anesthetic practice during pediatric renal transplantation. Material and Methods: The study consisted of a national survey about anesthetic practice during pediatric renal transplantation in France. French tertiary pediatric centers performing renal transplants were targeted, and one physician from each team was asked to complete the survey. The survey included patient data, preoperative assessment and optimization data, and intraoperative anesthesia data (drugs, ventilation, and hemodynamic interventions). Results: Twenty centers performing kidney transplantation were identified and contacted to complete the survey, and eight responded. Surveyed centers performed 96 of the 122 pediatric kidney transplantations performed in France in 2017 (79%). Centers consistently performed echocardiography and ultrasound examinations of the great veins preoperatively and consistently employed esophageal Doppler cardiac output estimation and vasopressors intraoperatively. All other practices were found to be heterogeneous. Central venous pressure was monitored in six centers, and dopamine was administered perioperatively in two centers. Conclusions: The current study provides a snapshot of the perioperative management of pediatric kidney transplantation in France. Results emphasize the need for both standardization of practice and awareness of recent evidence against the use of CVP monitoring and dopamine infusions. [ABSTRACT FROM AUTHOR]

The article discusses the French Emergency Medical Service and its positive impacts on the medical status of elderly patients in France. Statistics shows that of the 815 patients included 114 died on the scene, 632 admitted to the hospital, 500 were discharged alive, and 69 showed significant improvement in medical status. It is inferred that except for cardiac arrest the survival rate of patients for out-of-hospital emergency interventions was remarkably high.

Introduction: Malignant Hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that results as a hypermetabolic response to exposure to halogenated anaesthetic agents with or without depolarizing muscle relaxant (the direct role of depolarizing MR remains controversial). Early recognition of the signs of MH and specific treatment with Dantrium are critical to decrease morbidity and mortality of MH during anaesthesia. Moreover, the absence of specified patient's phenotypes, the dominant genetic transmission of MH and the high variability in genetic penetrance, require a specific knowledge of MH. [Extracted from the article]

Intensive care units are places which generate anxiety and stress, hospitalisations in these sectors often being sudden and a result of unexpected events. Furthermore, the family dynamics are often affected. This observation led to the creation of a surgical intensive care project at Caen university hospital, financed by the Fondation de France, which aims to ensure the parent-child bond is maintained., (Copyright © 2018. Publié par Elsevier Masson SAS.)