In-Hospital Clinical Features, Morbidity, and Mortality of Patients with Neurofibromatosis 1 in France: A Nationwide, Population-Based Retrospective Cohort Study.

Neurofibromatosis 1 (NF1) is a multisystem disease that can affect nearly every organ system. The aim of our study was to describe the in-hospital population with NF1 in France. We conducted a nationwide retrospective cohort study using the French hospital administrative database. A total of 11,425 patients with NF1 (53.4% female, 19,080 person years) were identified from January 2013 to December 2019. A total of 23% had at least one diagnosis of a comorbidity or NF1-associated complication or disease, and it was highest in the age group of 10-15 years. A total of 2,601 (22.8%) had a diagnosis of cancer. There were 366 (3.2%) in-hospital deaths, and we observed a standardized mortality ratio of 4.14 (95% confidence interval = 3.71-4.56), with a higher standardized mortality ratio in women and in the age group of 10-15 years. The standardized incident ratio (SIR) of cancer was 10.3 (95% confidence interval = 9.6-11.1). We observed high SIR values for cancer in childhood, with a decrease toward that of the general population by age 70 years. We observed high SIRs for NF1-associated cancers: CNS SIR of 195.4 (95% confidence interval = 172.2-220.9) and small intestine SIR of 102.9 (95% confidence interval = 71.7-143.2). The study provides a better understanding of the prognosis in people living with NF1.

Validation of New Methods of Using Simulated Whole-Body Movements as Implicit Indicators of Sound and Odor Preferences.

Would you get close to a stinky perfume bottle or to a loudspeaker producing noise? In this paper, we present two procedures that allowed us to assess the ability of auditory and olfactory cues to elicit automatic approach/avoidance reactions toward their sources. The procedures resulted from an adaptation of the Visual Approach/Avoidance by the Self Task (VAAST; Rougier et al., 2018), a task having the peculiarity of simulating approach/avoidance reactions by using visual feedback coming from the whole-body movements. In the auditory VAAST (Experiment 1), participants were instructed to move forward or backward from a loudspeaker that produced spoken words differentiated by their level of distortion and thus by their hedonic value. In the olfactory VAAST (Experiment 2), participants were asked to move forward or backward from a perfume bottle that delivered pleasant and unpleasant odors. We expected, consistent with the approach/avoidance compatibility effect, shorter latencies for approaching positive stimuli and avoiding negative stimuli. In both experiments, we found an effect of the quality of the emotional stimulus on forward actions of participants, with undistorted words and pleasant odors inducing faster forward movements compared with that for distorted words and unpleasant odors. Notably, our results further suggest that the VAAST can successfully be used with implicit instructions, i.e., without requiring participants to explicitly process the valence of the emotional stimulus (in Experiment 1) or even the emotional stimulus itself (in Experiment 2). The sensitivity of our procedures is analyzed and its potential in cross-modal and (contextualized) consumer research discussed.

Severe Attack of Henoch-Schönlein Purpura With Neurological Involvement During Adalimumab Treatment for Crohn's Disease.

Tumour necrosis factor-α [TNF-α] inhibitors have revolutionised the management of chronic inflammatory conditions. A number of cutaneous adverse events have been reported with TNF inhibition, including vasculitis. Most reactions are mild and rarely warrant treatment withdrawal. Here we describe a patient with Crohn's disease treated with adalimumab in whom severe multivisceral Henoch-Schönlein purpura developed, including neurological involvement, requiring definitive TNF blocker withdrawal.

Detection and prediction of driver drowsiness using artificial neural network models.

Not just detecting but also predicting impairment of a car driver's operational state is a challenge. This study aims to determine whether the standard sources of information used to detect drowsiness can also be used to predict when a given drowsiness level will be reached. Moreover, we explore whether adding data such as driving time and participant information improves the accuracy of detection and prediction of drowsiness. Twenty-one participants drove a car simulator for 110min under conditions optimized to induce drowsiness. We measured physiological and behavioral indicators such as heart rate and variability, respiration rate, head and eyelid movements (blink duration, frequency and PERCLOS) and recorded driving behavior such as time-to-lane-crossing, speed, steering wheel angle, position on the lane. Different combinations of this information were tested against the real state of the driver, namely the ground truth, as defined from video recordings via the Trained Observer Rating. Two models using artificial neural networks were developed, one to detect the degree of drowsiness every minute, and the other to predict every minute the time required to reach a particular drowsiness level (moderately drowsy). The best performance in both detection and prediction is obtained with behavioral indicators and additional information. The model can detect the drowsiness level with a mean square error of 0.22 and can predict when a given drowsiness level will be reached with a mean square error of 4.18min. This study shows that, on a controlled and very monotonous environment conducive to drowsiness in a driving simulator, the dynamics of driver impairment can be predicted.

Perception of Ultrasonic Switches Involves Large Discontinuity of the Mechanical Impedance.

The distinct tactile feedback provided by mechanical keyboards notifies users that their actions have been successfully recorded. The presence of these subtle yet informative tactile cues is one of the reasons why mechanical keyboards are still preferred to their virtual counterparts. An artificial sensation of pressing a mechanical switch can be produced by varying the coefficient of friction as the user is pressing down on a glass surface using ultrasonic vibration. We examined the factors involved in producing a vivid sensation of a stimulus by measuring the mechanical impedance, the frictional behavior of the fingertip and the perceptual thresholds. Subjects who experienced weaker sensations also showed a weaker sensitivity to friction modulation, which may in turn be attributable to the presence of a larger or a smaller than average impedance. In the second experiment, the user's finger impedance was measured during the click, and it was observed that the successful detection of the stimulus was correlated with the presence of considerable discontinuity in the mechanical impedance added to the plate by the finger. This discontinuity in the evolution of the impedance supports the idea that the skin is being reconfigured towards a new equilibrium state after the change in friction.