When Adolescent and Parents Disagree on Medical Plan, Who Gets to Decide?

Duchenne muscular dystrophy (DMD) is an X-linked autosomal recessive disease affecting 16 to 20 per 100 000 live births.1,2 It is characterized by progressive muscle weakness due to a defect in the dystrophin gene. It typically leads to loss of ambulation by age 8 to 14 years,1 followed by cardiomyopathy and respiratory failure. Historically, adolescents with DMD have died at ∼20 years of age.1–3 As respiratory compromise occurs, patients are supported with noninvasive ventilation (eg, nasal bilevel positive airway pressure).3–6 When this becomes unsuccessful, patients may be candidates for tracheostomy; this often happens in the second or third decade of life.7 The decision of whether to proceed with tracheostomy is complicated and is most often left to the patient and family. Family members do not always agree. We present a case in which acute illness forced a minor and his family to face this decision earlier than is typical. The adolescent desired a tracheostomy to extend his life. The parents did not believe that a tracheostomy was in his best interest and felt that comfort care was the most appropriate approach. Experts comment on the ethical issues raised by medical decision-making in cases involving adolescents and life-and-death decisions

The performance of long vs. short questionnaire-based measures of depression, anxiety, and psychological distress among UK adults: A comparison of the patient health questionnaires, generalized anxiety disorder scales, malaise inventory, and Kessler scales

It is often important to minimise the time participants in social science studies spend on completing questionnaire-based measures, reducing response burden, and increasing data quality. Here, we investigated the performance of the short versions of some widely used depression, anxiety, and psychological distress scales and compared them to the performance of longer versions of these scales (PHQ-2 vs PHQ-9, GAD-2 vs GAD-7, Malaise-3 vs Malaise-9, K6 vs K10). Across a sample of UK adults (N = 987, ages 18-86), we tested the existing factor structure and accuracy of the scales through confirmatory factor analyses and exploration of the total information functions, observing adequate model fit indices across the measures. Measurement invariance was tested across birth sex and age groups to explore whether any differences in measurement properties or measurement bias may exist, finding support for the invariance of most measures. We conducted bivariate correlations across the measures as a way of obtaining evidence of the equivalence in the rank-ordering of short vs long scales. The results followed a similar pattern across the young adult subsample (N = 375, ages 18-39) as in the overall sample. Overall, these results indicate that the short forms of the tested scales may perform similarly to the full versions. Where brevity is important, researchers may opt to use the shorter versions of the scales based on these data

Validation of modelling the radiation exposure due to solar particle events at aircraft altitudes

Dose assessment procedures for cosmic radiation exposure of aircraft crew have been introduced in most European countries in accordance with the corresponding European directive and national regulations. However, the radiation exposure due to solar particle events is still a matter of scientific research. Here we describe the European research project CONRAD, WP6, Subgroup-B, about the current status of available solar storm measurements and existing models for dose estimation at flight altitudes during solar particle events leading to ground level enhancement (GLE). Three models for the numerical dose estimation during GLEs are discussed. Some of the models agree with limited experimental data reasonably well. Analysis of GLEs during geomagnetically disturbed conditions is still complex and time consuming. Currently available solar particle event models can disagree with each other by an order of magnitude. Further research and verification by on-board measurements is still neede

Survey on solar X-ray flares and associated coherent radio emissions

The radio emission during 201 X-ray selected solar flares was surveyed from 100 MHz to 4 GHz with the Phoenix-2 spectrometer of ETH Zurich. The selection includes all RHESSI flares larger than C5.0 jointly observed from launch until June 30, 2003. Detailed association rates of radio emission during X-ray flares are reported. In the decimeter wavelength range, type III bursts and the genuinely decimetric emissions (pulsations, continua, and narrowband spikes) were found equally frequently. Both occur predominantly in the peak phase of hard X-ray (HXR) emission, but are less in tune with HXRs than the high-frequency continuum exceeding 4 GHz, attributed to gyrosynchrotron radiation. In 10% of the HXR flares, an intense radiation of the above genuine decimetric types followed in the decay phase or later. Classic meter-wave type III bursts are associated in 33% of all HXR flares, but only in 4% they are the exclusive radio emission. Noise storms were the only radio emission in 5% of the HXR flares, some of them with extended duration. Despite the spatial association (same active region), the noise storm variations are found to be only loosely correlated in time with the X-ray flux. In a surprising 17% of the HXR flares, no coherent radio emission was found in the extremely broad band surveyed. The association but loose correlation between HXR and coherent radio emission is interpreted by multiple reconnection sites connected by common field lines.Comment: Solar Physics, in pres

FAS-dependent cell death in α-synuclein transgenic oligodendrocyte models of multiple system atrophy

Multiple system atrophy is a parkinsonian neurodegenerative disorder. It is cytopathologically characterized by accumulation of the protein p25α in cell bodies of oligodendrocytes followed by accumulation of aggregated α-synuclein in so-called glial cytoplasmic inclusions. p25α is a stimulator of α-synuclein aggregation, and coexpression of α-synuclein and p25α in the oligodendroglial OLN-t40-AS cell line causes α-synuclein aggregate-dependent toxicity. In this study, we investigated whether the FAS system is involved in α-synuclein aggregate dependent degeneration in oligodendrocytes and may play a role in multiple system atrophy. Using rat oligodendroglial OLN-t40-AS cells we demonstrate that the cytotoxicity caused by coexpressing α-synuclein and p25α relies on stimulation of the death domain receptor FAS and caspase-8 activation. Using primary oligodendrocytes derived from PLP-α-synuclein transgenic mice we demonstrate that they exist in a sensitized state expressing pro-apoptotic FAS receptor, which makes them sensitive to FAS ligand-mediated apoptosis. Immunoblot analysis shows an increase in FAS in brain extracts from multiple system atrophy cases. Immunohistochemical analysis demonstrated enhanced FAS expression in multiple system atrophy brains notably in oligodendrocytes harboring the earliest stages of glial cytoplasmic inclusion formation. Oligodendroglial FAS expression is an early hallmark of oligodendroglial pathology in multiple system atrophy that mechanistically may be coupled to α-synuclein dependent degeneration and thus represent a potential target for protective intervention