The trajectory to diagnosis with pulmonary arterial hypertension: a qualitative study

Objectives To investigate the patient's experience of the trajectory to receiving a diagnosis of pulmonary arterial hypertension (PAH) and inform the provision of care for this patient group. Design Qualitative study using in-depth one-to-one interviews and pictorial representations. Data were analysed using thematic analysis. Setting Participants were interviewed in their own homes across England. Participants 30 patients with a diagnosis of pulmonary hypertension (18 participants were women, mean age 56 and range 26-80 02years and time since diagnosis ranged from a few months to more than 12 02years) participated. Results All participants, regardless of the time since diagnosis, vividly described the process from manifestation of symptoms to receiving a confirmed diagnosis. The authors present data using three major themes: (i) making sense of symptoms, (ii) process of elimination and (iii) being diagnosed with PAH. Making sense of symptoms represented an early period of perseverance 14people tried to carry-on as usual despite 18unexplained breathlessness 19. As time progressed, this period was punctuated by critical events that triggered seeking medical advice. Once medical contact had been made, patients described a period of 18elimination 19 and convoluted contact with the medical profession. Dyspnoea misdiagnosis was a key factor that delayed the PAH diagnosis. Diagnosis disclosure by some medical professionals was also viewed as lacking empathy. More positive experiences were relayed when the medical team disclosing the diagnosis acknowledged previous limitations. Conclusions A lack of awareness of this illness from both the sufferer themselves and the medical profession emerged as a central theme and led to prolonged periods of being misdiagnosed. The application of a diagnostic pathway for unexplained dyspnoea that alerts practitioners to rare conditions could expedite the process of correct diagnosis

Certainty Closure: Reliable Constraint Reasoning with Incomplete or Erroneous Data

Constraint Programming (CP) has proved an effective paradigm to model and solve difficult combinatorial satisfaction and optimisation problems from disparate domains. Many such problems arising from the commercial world are permeated by data uncertainty. Existing CP approaches that accommodate uncertainty are less suited to uncertainty arising due to incomplete and erroneous data, because they do not build reliable models and solutions guaranteed to address the user's genuine problem as she perceives it. Other fields such as reliable computation offer combinations of models and associated methods to handle these types of uncertain data, but lack an expressive framework characterising the resolution methodology independently of the model. We present a unifying framework that extends the CP formalism in both model and solutions, to tackle ill-defined combinatorial problems with incomplete or erroneous data. The certainty closure framework brings together modelling and solving methodologies from different fields into the CP paradigm to provide reliable and efficient approches for uncertain constraint problems. We demonstrate the applicability of the framework on a case study in network diagnosis. We define resolution forms that give generic templates, and their associated operational semantics, to derive practical solution methods for reliable solutions.Comment: Revised versio

Episodic excursions of low-mass protostars on the Hertzsprung-Russell diagram

Following our recent work devoted to the effect of accretion on the pre-main-sequence evolution of low-mass stars, we perform a detailed analysis of episodic excursions of low-mass protostars in the Hertzsprung-Russell (H-R) diagram triggered by strong mass accretion bursts typical of FU Orionis-type objects (FUors). These excursions reveal themselves as sharp increases in the stellar total luminosity and/or effective temperature of the protostar and can last from hundreds to a few thousands of years, depending on the burst strength and characteristics of the protostar. During the excursions, low-mass protostars occupy the same part of the H-R diagram as young intermediate-mass protostars in the quiescent phase of accretion. Moreover, the time spent by low-mass protostars in these regions is on average a factor of several longer than that spent by the intermediate-mass stars in quiescence. During the excursions, low-mass protostars pass close to the position of most known FUors in the H-R diagram, but owing to intrinsic ambiguity the model stellar evolutionary tracks are unreliable in determining the FUor properties. We find that the photospheric luminosity in the outburst state may dominate the accretion luminosity already after a few years after the onset of the outburst, meaning that the mass accretion rates of known FUors inferred from the bolometric luminosity may be systematically overestimated, especially in the fading phase.Comment: 15 pages, 12 figure

Massive stars and the energy balance of the interstellar medium. II. The 35 solar mass star and a solution to the "missing wind problem"

We continue our numerical analysis of the morphological and energetic influence of massive stars on their ambient interstellar medium for a 35 solar mass star that evolves from the main sequence through red supergiant and Wolf-Rayet phases, until it ultimately explodes as a supernova. We find that structure formation in the circumstellar gas during the early main-sequence evolution occurs as in the 60 solar mass case but is much less pronounced because of the lower mechanical wind luminosity of the star. Since on the other hand the shell-like structure of the HII region is largely preserved, effects that rely on this symmetry become more important. At the end of the stellar lifetime 1% of the energy released as Lyman continuum radiation and stellar wind has been transferred to the circumstellar gas. From this fraction 10% is kinetic energy of bulk motion, 36% is thermal energy, and the remaining 54% is ionization energy of hydrogen. The sweeping up of the slow red supergiant wind by the fast Wolf-Rayet wind produces remarkable morphological structures and emission signatures, which are compared with existing observations of the Wolf-Rayet bubble S308. Our model reproduces the correct order of magnitude of observed X-ray luminosity, the temperature of the emitting plasma as well as the limb brightening of the intensity profile. This is remarkable, because current analytical and numerical models of Wolf-Rayet bubbles fail to consistently explain these features. A key result is that almost the entire X-ray emission in this stage comes from the shell of red supergiant wind swept up by the shocked Wolf-Rayet wind rather than from the shocked Wolf-Rayet wind itself as hitherto assumed and modeled. This offers a possible solution to what is called the ``missing wind problem'' of Wolf-Rayet bubbles.Comment: 52 pages, 20 figures, 2 tables, accepted for publication in the Astrophysical Journa

HST/WFPC2 and VLT/ISAAC observations of PROPLYDS in the giant HII region NGC 3603

We report the discovery of three proplyd-like structures in the giant HII region NGC 3603. The emission nebulae are clearly resolved in narrow-band and broad-band HST/WFPC2 observations in the optical and broad-band VLT/ISAAC observations in the near-infrared. All three nebulae are tadpole shaped, with the bright ionization front at the head facing the central cluster and a fainter ionization front around the tail pointing away from the cluster. Typical sizes are 6,000 A.U. x 20,000 A.U. The nebulae share the overall morphology of the proplyds (``PROto PLanetarY DiskS'') in Orion, but are 20 to 30 times larger in size. Additional faint filaments located between the nebulae and the central ionizing cluster can be interpreted as bow shocks resulting from the interaction of the fast winds from the high-mass stars in the cluster with the evaporation flow from the proplyds. The striking similarity of the tadpole shaped emission nebulae in NGC 3603 to the proplyds in Orion suggests that the physical structure of both types of objects might be the same. We present 2D radiation hydrodynamical simulations of an externally illuminated star-disk-envelope system, which was still in its main accretion phase when first exposed to ionizing radiation from the central cluster. The simulations reproduce the overall morphology of the proplyds in NGC 3603 very well, but also indicate that mass-loss rates of up to 10^-5 Mo/yr are required in order to explain the size of the proplyds. (abbreviated)Comment: 10 pages, 4 Postscript figures, uses emulateapj.sty and psfig.tex. Astronomical Journal, in press (January 2000 issue

The patient experience of pulmonary hypertension : a large cross-sectional study of UK patients

Background Pulmonary Hypertension Association UK (PHA-UK) is the only charity in the UK especially for people affected by pulmonary hypertension (PH). To better understand the impact of PH on patients and carers beyond clinical symptoms, the PHA-UK carried out a cross-sectional survey on the effect of PH on daily living, along with a follow-up survey assessing the financial impact of PH. Methods This is a descriptive cross-sectional survey of adult patients with PH in the UK. A quantitative survey of four key topics (time to diagnosis, quality of life [QoL], financial impact and specialist treatment), was made available to PHA-UK members and patients on PH therapy, with a follow-up financial impact survey sent to those responders who agreed to be contacted further. Data collection was carried out in January and February 2017 for the main survey, and November and December 2017 for the financial impact survey. Results The main survey was completed by 567 individuals, and the financial follow-up survey by 171. Mean age of responders was 69 ± 17 years with 70% female. 60% of respondents said PH had a major impact on their QoL, with 45% reporting that treatment and management improves their QoL ‘a lot’. The time between first experiencing symptoms and diagnosis was ≥1 year for 48% of patients, with 40% seeing 4+ doctors before diagnosis. 63% of patients reported financial worries. Patients in part-time and full-time work reported the greatest financial burden, with a 13 and 33% fall in monthly income respectively. Patients had positive experiences of treatment in specialist centres, with 62% rating their care ‘excellent’, and 92% saying they preferred travelling to a specialist centre rather than seeing a local non-specialist. Conclusions This study reports the largest UK survey exploring issues affecting patients with PH. The study shows that despite the availability of new therapies, patients are still experiencing delays prior to diagnosis, and experiencing both emotional and financial impacts from the disease. By identifying the areas patients find most important in their treatment, this research can inform future care policies and long-term management to support patients living with PH and their families

emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension.

The aim of this study was to develop a measure of the impact of pulmonary hypertension (PH) on health-related quality of life (HRQoL) as there is a need for a short, validated instrument that can be used in routine clinical practice. Interviews were conducted with 30 PH patients to derive 32 statements, which were presented as a semantic differential six-point scale (0–5), with contrasting adjectives at each end. This item list was completed by patients attending PH clinics across the UK and Ireland. Rasch analysis was applied to identify items fitting a uni-dimensional model. 226 patients (mean age 55.6±14 years; 70% female) with PH (82% had pulmonary arterial hypertension) completed the study questionnaires. 10 of the 32 items demonstrated fit to the Rasch model (Chi-squared 16; p>0.05) and generated the emPHasis-10 questionnaire. Test–retest (intraclass correlation coefficient 0.95, n=33) and internal consistency (Chronbach’s α=0.9) were strong. emPHasis-10 scores correlated consistently with other relevant measures and discriminated subgroups of patients stratified by World Health Organization functional class (ANOVA F=1.73; p<0.001). The emPHasis-10 is a short questionnaire for assessing HRQoL in pulmonary arterial hypertension. It has excellent measurement properties and is sensitive to differences in relevant clinical parameters. It is freely available for clinical and academic use

Toward Understanding Massive Star Formation

Although fundamental for astrophysics, the processes that produce massive stars are not well understood. Large distances, high extinction, and short timescales of critical evolutionary phases make observations of these processes challenging. Lacking good observational guidance, theoretical models have remained controversial. This review offers a basic description of the collapse of a massive molecular core and a critical discussion of the three competing concepts of massive star formation: - monolithic collapse in isolated cores - competitive accretion in a protocluster environment - stellar collisions and mergers in very dense systems We also review the observed outflows, multiplicity, and clustering properties of massive stars, the upper initial mass function and the upper mass limit. We conclude that high-mass star formation is not merely a scaled-up version of low-mass star formation with higher accretion rates, but partly a mechanism of its own, primarily owing to the role of stellar mass and radiation pressure in controlling the dynamics.Comment: 139 pages, 18 figures, 5 tables, glossar

Simultaneous Continuation of Infinitely Many Sinks Near a Quadratic Homoclinic Tangency

We prove that the $C^3$ diffeomorphisms on surfaces, exhibiting infinitely many sinksnear the generic unfolding of a quadratic homoclinic tangency of a dissipative saddle, can be perturbed along an infinite dimensional manifold of $C^3$ diffeomorphisms such that infinitely many sinks persist simultaneously. On the other hand, if they are perturbed along one-parameter families that unfold generically the quadratic tangencies, then at most a finite number of those sinks have continuation