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An improved understanding of diagnostic and treatment practices for patients with rare primary mitochondrial disorders can support benchmarking against guidelines and establish priorities for evaluative research. We aimed to describe physician care for patients with mitochondrial diseases in Canada, including variation in care.
We conducted a cross-sectional survey of Canadian physicians involved in the diagnosis and/or ongoing care of patients with mitochondrial diseases. We used snowball sampling to identify potentially eligible participants, who were contacted by mail up to five times and invited to complete a questionnaire by mail or internet. The questionnaire addressed: personal experience in providing care for mitochondrial disorders; diagnostic and treatment practices; challenges in accessing tests or treatments; and views regarding research priorities.
We received 58 survey responses (52% response rate). Most respondents (83%) reported spending 20% or less of their clinical practice time caring for patients with mitochondrial disorders. We identified important variation in diagnostic care, although assessments frequently reported as diagnostically helpful (e.g., brain magnetic resonance imaging, MRI/MR spectroscopy) were also recommended in published guidelines. Approximately half (49%) of participants would recommend “mitochondrial cocktails” for all or most patients, but we identified variation in responses regarding specific vitamins and cofactors. A majority of physicians recommended studies on the development of effective therapies as the top research priority.
While Canadian physicians’ views about diagnostic care and disease management are aligned with published recommendations, important variations in care reflect persistent areas of uncertainty and a need for empirical evidence to support and update standard protocols.
The discovery of the first electromagnetic counterpart to a gravitational wave signal has generated follow-up observations by over 50 facilities world-wide, ushering in the new era of multi-messenger astronomy. In this paper, we present follow-up observations of the gravitational wave event GW170817 and its electromagnetic counterpart SSS17a/DLT17ck (IAU label AT2017gfo) by 14 Australian telescopes and partner observatories as part of Australian-based and Australian-led research programs. We report early- to late-time multi-wavelength observations, including optical imaging and spectroscopy, mid-infrared imaging, radio imaging, and searches for fast radio bursts. Our optical spectra reveal that the transient source emission cooled from approximately 6 400 K to 2 100 K over a 7-d period and produced no significant optical emission lines. The spectral profiles, cooling rate, and photometric light curves are consistent with the expected outburst and subsequent processes of a binary neutron star merger. Star formation in the host galaxy probably ceased at least a Gyr ago, although there is evidence for a galaxy merger. Binary pulsars with short (100 Myr) decay times are therefore unlikely progenitors, but pulsars like PSR B1534+12 with its 2.7 Gyr coalescence time could produce such a merger. The displacement (~2.2 kpc) of the binary star system from the centre of the main galaxy is not unusual for stars in the host galaxy or stars originating in the merging galaxy, and therefore any constraints on the kick velocity imparted to the progenitor are poor.
Offspring of mothers with depression are at heightened risk of psychiatric disorder. Many mothers with depression have comorbid psychopathology. How these co-occurring problems affect child outcomes has rarely been considered.
To consider whether the overall burden of co-occurring psychopathology in mothers with recurrent depression predicts new-onset psychopathology in offspring.
Mothers with recurrent depression and their adolescent offspring (9–17 years at baseline) were assessed in 2007 and on two further occasions up to 2011. Mothers completed questionnaires assessing depression severity, anxiety, alcohol problems and antisocial behaviour. Psychiatric disorder in offspring was assessed using the Child and Adolescent Psychiatric Assessment.
The number of co-occurring problems in mothers (0, 1 or 2+) predicted new-onset offspring disorder (odds ratio (OR) = 1.80, 95% CI 1.17–2.77, P = 0.007). Rates varied from 15.7 to 34.8% depending on the number of co-occurring clinical problems. This remained significant after controlling for maternal depression severity (OR = 1.73, 95% CI 1.03–2.89, P = 0.040).
The burden of co-occurring psychopathology among mothers with recurrent depression indexes increased risk of future onset of psychiatric disorder for offspring. This knowledge can be used in targeting preventive measures in children at high risk of psychiatric disorder.
As the remnant of Supernova (SN) 1987A has been getting brighter over time, new observations at high frequencies have allowed imaging of the radio emission at unprecedented detail. We present a new radio image at 44 GHz of the supernova remnant (SNR), derived from observations performed with the Australia Telescope Compact Array (ATCA) in 2011. The diffraction-limited image has a resolution of 349×225 mas, which is the highest achieved to date in high-dynamic range images of the SNR. We also present a new image at 18 GHz, also derived from ATCA observations performed in 2011, which is super-resolved to 0″.25. The new 44 and 18 GHz images yield the first high-resolution spectral index map of the remnant. The comparison of the 44 GHz image with contemporaneous X-ray and Hα observations allows further investigations of the nature of the remnant asymmetry and sheds more light into the progenitor hypotheses and SN explosion. In light of simple free-free absorption models, we discuss the likelihood of detecting at 44 GHz the possible emission originating from a pulsar wind nebula (PWN) or a compact source in the centre of the remnant.
Supernovae and their remnants are believed to be prodigious sources of Galactic cosmic rays and interstellar dust. Understanding the mechanisms behind their surprisingly high production rate is helped by the study of nearby young supernova remnants. There has been none better in modern times than SN1987A, for which radio observations have been made for over a quarter of a century. We review extensive observations made with the Australia Telescope Compact Array (ATCA) at centimetre wavelengths. Emission at frequencies from 1 to 100 GHz is dominated by synchrotron radiation from an outer shock front which has been growing exponentially in strength from day 3000, and is currently sweeping around the circumstellar ring at about 4000 km s−1. Three dimensional models of the propagation of the shock into the circumstellar medium are able to reproduce the main observational features of the remnant, and their evolution. We find that up to 4% of the electrons encountered by the shock are accelerated to relativistic energies. High-frequency ALMA observations will break new ground in the understanding of dust and molecule production.
Johann Wolfgang von Goethe's pathbreaking epistolary novel, Die Leiden des jungen Werther (1774, 2nd [rev.] ed. 1787) has been the focus of an enormous amount of scholarly attention. A recent analysis by Bruce Duncan of more than two centuries of Werther criticism, Goethe's “Werther” and the Critics (2005), makes manifest the wide variety of critical approaches to this extremely rich text. Duncan discusses, among other things, contemporary late eighteenth-century reactions to Werther, biographical, religious, psychological, and political approaches to Goethe's novel, as well as interpretations of Werther that focus on reading, writing, gender, and/or sexuality. The literary critic Michael Bell has determined that critical responses to this novel generally reflect one of two divergent modes of understanding Werther: readers tend to view Werther as either “a romantic tragedy” or “a neurotic casehistory,” depending on whether they are reading in an “identificatory” or an “ironical” manner. Bell views these trends as equally justifiable yet mutually exclusive methods of reading the text. Bell's understanding of the novel's critical reception explains not only the intense response, both enthusiastic and condemnatory, from eighteenth-century readers, but also the great number of scholarly contributions that have read Werther as a pathography. The tradition of reading Werther as a pathography goes back as far as the contemporary reception of the novel in the late eighteenth century, as in, for example, Friedrich Nicolai's Freuden des jungen Werthers: Leiden und Freuden Werthers des Mannes (1775), a text which will be discussed later in this essay.
Christian Fürchtegott Gellert (1715–69) exerted a tremendous influence on his contemporaries throughout eighteenth-century Europe, reaching into many levels of society, and at his death, Leipzig authorities had to declare his grave off-limits to visitors, as the sheer number of his mourners had begun to take a toll on the cemetery and Gellert's enthusiastic fans had taken to filling reliquaries with earth from his grave site. Gellert's sentimental comedy Die zärtlichen Schwestern (The tender sisters, 1747) was influential as well. The play was published in five editions throughout the eighteenth century, translated into four European languages, and performed many times on the stage after its premiere, which was put on by the Neuber troupe. The play was well received by contemporaries such as Christian Heinrich Schmid and Johann Georg Hamann.
Gellert, like Gottsched, was profoundly interested in morality. He earned his reputation as a writer of fables, a genre devoted to the moral education of its audience, and he published a series of lectures on morality, including moralische Charaktere, or moral vignettes, which he had given as a professor at the University of Leipzig. Gellert, like Gottsched, traces immorality to the individual's lack of success in controlling his or her passions and to the way in which the passions cloud reason, the faculty that enables one to make the correct moral choices. One of the most prominent and most dangerous of the passions, according to Gellert, is sexual desire.