Search

While previous cometary missions were only limited to flybys, the groundbreaking ESA/Rosetta mission escorted comet 67P for two years. This enabled for the first time to make in situ measurements of the evolution of a comet's plasma environment. On one hand, low time resolution electron densities are calculated using the plasma frequency extracted from the mutual impedance spectra measured by the RPC-Mutual Impedance Probe (MIP). On the other hand, high time resolution fluctuations of spacecraft potential and ion current are measured by the RPC-Langmuir Probe (LAP). Thus, we perform a MIP-LAP cross-calibration using a spacecraft charging model to retrieve high time resolution electron density fluctuations throughout the entire mission (whenever both the required MIP and LAP operations and data allow). This new dataset will be available to the scientific community on the ESA/PSA around Summer 2019, however it is already available for specific cases of interest. Here, we make use of these data, together with RPC-MAG magnetic field measurements, to investigate in detail the properties of the low-frequency so-called "singing comet" waves observed in the plasma environment of 67P, making use of a change in the wave activity before and during a cometary brightness outburst on February 19, 2016.

The compromise of quality of life in Huntington's disease is a major issue, both for individuals with the disease as well as for their caregivers. The International Parkinson and Movement Disorder Society commissioned a review of the use and clinimetric validation status of measures used in Huntington's disease to assess aspects related with quality of life and to make recommendations on their use following standardized criteria. We included both patient-centered measures (patient health-related quality-of-life measures) and caregiver-centered measures (caregiver quality-of-life measures). After conducting a systematic literature search, we included 12 measures of patient health-related quality of life and 2 measures of caregiver quality of life. Regarding patient-centered measures, the Medical Outcomes Study 36-Item Short-Form Health Survey is "recommended" as a generic assessment of health-related quality of life in patients with Huntington's disease. The 12-Item Short Form Health Survey, the Sickness Impact Profile, the 12-item World Health Organization Disability Assessment Schedule, and the Huntington's Disease Health-Related Quality of Life questionnaire are "suggested." No caregiver-centered quality-of-life measure obtained a "recommended" status. The Alzheimer's Carer's Quality of Life Inventory and the Huntington's Disease Quality of Life Battery for Carers are "suggested." Recognizing that the assessment of patient health-related quality of life can be challenging in Huntington's disease, as patients may lack insight and there is insufficient clinimetric testing of these scales, the committee concluded that further validation of currently available health-related quality-of-life measures should be undertaken, namely, those Huntington's disease-specific health-related quality-of-life measures that have recently been reported and used. (c) 2018 International Parkinson and Movement Disorder Society.

Limitation of functional ability is a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the appraisal of the use and clinimetric properties of clinical measures of functional ability that have been applied in HD studies and trials to date, to make recommendations regarding their use based on standardized criteria. After a systematic literature search, we included a total of 29 clinical measures grouped into two categories: (1) performance-based measures (e.g., balance, walking, and reaching/grasping), and (2) rating scales. Three performance-based measures are rated as "recommended": the Tinetti Mobility Test for screening of fall risk and for severity assessment of mobility in patients with manifest HD (up to stage III); the Berg Balance Scale for severity of balance impairment; and the Six-Minute Walk Test for assessment of walking endurance (severity) in HD subjects with preserved ambulation. No rating scale targeting functional ability reached a "recommended" status either for screening or severity measurement. The main challenges identified in this review include applying widely accepted conceptual frameworks to the identified measures, the lack of validation of clinical measures to detect change over time, and absence of validated measures for upper limb function. Furthermore, measures of capacity or ability to perform activities of daily living had ceiling effects in people with early and pre-manifest HD. We recommend that the MDS prioritize the development of new scales that capture small, but meaningful changes in function over time for outcome assessment in clinical trials, particularly in earlier stages of HD.

Due to age dependent organ manifestation, diagnosis of Marfan syndrome (MFS) is a challenge, especially in childhood. It is important to identify children at risk of MFS as soon as possible to direct those to appropriate treatment but also to avoid stigmatization due to false diagnosis. We published the Kid-Short Marfan Score (Kid-SMS) in 2012 to stratify the pre-test probability of MFS in childhood. Hence we now evaluate the predictive performance of Kid-SMS in a new cohort of children. We prospectively investigated 106 patients who were suspected of having MFS. At baseline, children were examined according to Kid-SMS. At baseline and follow-up visit, diagnosis of MFS was established or rejected using standard current diagnostic criteria according to the revised Ghent Criteria (Ghent-2). At baseline 43 patients were identified with a risk of MFS according to Kid-SMS whereas 21 patients had Ghent-2 diagnosis of MFS. Sensitivity was 100%, specificity 77%, negative predictive value 100% and Likelihood ratio of Kid-SMS 4.3. During follow-up period, three other patients with a stratified risk for MFS were diagnosed according to Ghent-2. We confirm very good predictive performance of Kid-SMS with excellent sensitivity and negative predictive value but restricted specificity. Kid-SMS avoids stigmatization due to diagnosis of MFS and thus restriction to quality of life. Especially outpatient pediatricians and pediatric cardiologists can use it for primary assessment.

International audience; During two years, the groundbreaking ESA/Rosetta mission was able to escort comet 67P where previous cometary missions were only limited to flybys. This enabled for the first time to make in-situ measurements of the evolution of a comet's plasma environment. The density and temperature measured by Rosetta are derived from RPC-Mutual Impedance Probe (MIP) and RPC-Langmuir Probe (LAP). On one hand, low time resolution electron density are calculated using the plasma frequency extracted from the MIP mutual impedance spectra. On the other hand, high time resolution density fluctuations are estimated from the spacecraft potential measured by LAP. In this study, using a simple spacecraft charging model, we perform a cross-calibration of MIP plasma density and LAP spacecraft potential variations to obtain high time resolution measurements of the electron density. These results are also used to constrain the electron temperature. Then we make use of these new dataset, together with RPC-MAG magnetic field measurements, to investigate for the first time the compressibility and the correlations between plasma and magnetic field variations, for both singing comet waves and steepened waves observed, respectively during low and high cometary outgassing activity, in the plasma environment of comet 67P.

The magnetometer onboard ESA's Rosetta orbiter detected its highest magnetic field magnitude of 250nT in July 2015, close to perihelion. This magnitude was an enhancement of a factor of five compared to normal values, which makes this the highest interplanetary magnetic field ever measured. We have examined the solar wind conditions at the time and found that a corotating interaction region (CIR), accompanied by a fast flow is the trigger for this unusual event. Because Rosetta does not have solar wind observations during the comet's active phase, we use ENLIL simulations as well as observations at Earth and Mars to constrain the solar wind parameters at the comet. Using a simple model for the magnetic field pile-up we can trace back the field in the coma to corresponding structures in the CIR. The large field is accompanied by a dramatic increase in electron and ion fluxes and energies. However, the electrons and ions in the field of view are not, as expected, increasing at the same time, instead the electrons follow the magnetic field, while the ion density increase is delayed. This is seen as evidence of the kinetic behaviour of the ions as opposed to a magnetized electron fluid. Combining the information on the plasma, we are able to identify at least three different regions in the plasma that have fundamentally different parameters. This allows us to separate the solar wind influence from the comet's effects on the plasma, a problem that is usually not solvable without a spacecraft monitoring the solar wind at the comet.

International audience; We discuss observations of the journey throughout the Solar System of a large interplanetary coronal mass ejection (ICME) that was ejected at the Sun on 14 October 2014. The ICME hit Mars on 17 October, as observed by the Mars Express, MAVEN, Mars Odyssey and MSL missions, 44 hours before the encounter of the planet with the Siding-Spring comet, for which the space weather context is provided. It reached comet 67P/Churyumov-Gerasimenko, which was perfectly aligned with the Sun and Mars at 3.1 AU, as observed by Rosetta on 22 October. The ICME was also detected by STEREO-A on 16 October at 1 AU, and by Cassini in the solar wind around Saturn on the 12 November at 9.9 AU. Fortuitously, the New Horizons spacecraft was also aligned with the direction of the ICME at 31.6 AU. We investigate whether this ICME has a non-ambiguous signature at New Horizons. A potential detection of this ICME by Voyager-2 at 110-111 AU is also discussed. The multi-spacecraft observations allow the derivation of certain properties of the ICME, such as its large angular extension of at least 116°, its speed as a function of distance, and its magnetic field structure at four locations from 1 to 10 AU. Observations of the speed data allow two different solar wind propagation models to be validated. Finally, we compare the Forbush decreases (transient decreases followed by gradual recoveries in the galactic cosmic ray intensity) due to the passage of this ICME at Mars, comet 67P and Saturn.

We discuss observations of the journey throughout the Solar System of a large interplanetary coronal mass ejection (ICME) that was ejected at the Sun on 14 October 2014. The ICME hit Mars on 17 October, as observed by the Mars Express, Mars Atmosphere and Volatile EvolutioN Mission (MAVEN), Mars Odyssey, and Mars Science Laboratory (MSL) missions, 44h before the encounter of the planet with the Siding-Spring comet, for which the space weather context is provided. It reached comet 67P/Churyumov-Gerasimenko, which was perfectly aligned with the Sun and Mars at 3.1 AU, as observed by Rosetta on 22 October. The ICME was also detected by STEREO-A on 16 October at 1 AU, and by Cassini in the solar wind around Saturn on the 12 November at 9.9AU. Fortuitously, the New Horizons spacecraft was also aligned with the direction of the ICME at 31.6 AU. We investigate whether this ICME has a nonambiguous signature at New Horizons. A potential detection of this ICME by Voyager 2 at 110-111 AU is also discussed. The multispacecraft observations allow the derivation of certain properties of the ICME, such as its large angular extension of at least 116 degrees, its speed as a function of distance, and its magnetic field structure at four locations from 1 to 10 AU. Observations of the speed data allow two different solar wind propagation models to be validated. Finally, we compare the Forbush decreases (transient decreases followed by gradual recoveries in the galactic cosmic ray intensity) due to the passage of this ICME at Mars, comet 67P, and Saturn.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.