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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.
Pikeperch (Sander lucioperca) is a highly valuable fish in Europe. However, development of aquaculture of pikeperch is highly limited due to seasonality of production. This can be overcome by the controlled reproduction of domesticated fish. The first steps of domestication process may induce changes at anatomical, physiological and molecular levels, thereby affecting a variety of biological functions. While there is abundant literature on their effects on stress and growth for example, these effects on reproduction received limited attention notably in pikeperch, a promising candidate for the development of aquaculture. To answer the question of this life-history effect on pikeperch’s reproduction, we compared two groups (weight: 1 kg) originated from Czech Republic and with the same domestication level (F0). The first group was a recirculating aquatic system cultured one (2 years, previously fed with artificial diet, never exposed to natural changes in temperature/photoperiod conditions) and the second one was a pond cultured group (3 to 4 years, bred under natural feeding and temperature/photoperiod). The wild group successfully spawned, while the farmed one did not spawn at all. During the program, gonadosomatic indexes of both males and females were significantly higher for the wild fish, as well as the sexual steroids. Gene expression analysis revealed significantly lower LH transcript levels at the pituitary level for the farmed females and lower FSH transcript levels at the pituitary level for the males. In conclusion this study showed that the previous rearing conditions (e.g. culture system, age, diet, etc.) alter the further progress of gametogenesis and the reproductive performances in response to controlled photothermal program for both sexes in pikeperch.
Populations of the White-rumped Vulture Gyps bengalensis, Indian Vulture G. indicus and Slender-billed Vulture G. tenuirostris declined rapidly during the mid-1990s all over their ranges in the Indian subcontinent because of poisoning due to veterinary use of the non-steroidal anti-inflammatory drug diclofenac. This paper reports results from the latest in a series of road transect surveys conducted across northern, central, western and north-eastern India since the early 1990s. Results from the seven comparable surveys now available were analysed to estimate recent population trends. Populations of all three species of vulture remained at a low level. The previously rapid decline of White-rumped Vulture has slowed and may have reversed since the ban on veterinary use of diclofenac in India in 2006. A few thousand of this species, possibly up to the low tens of thousands, remained in India in 2015. The population of Indian Vulture continued to decline, though probably at a much slower rate than in the 1990s. This remains the most numerous of the three species in India with about 12,000 individuals in 2015 and a confidence interval ranging from a few thousands to a few tens of thousands. The trend in the rarest species, Slender-billed Vulture, which probably numbers not much more than 1,000 individuals in India, cannot be determined reliably.
The objective of this study was to examine the religious/spiritual beliefs of followers of the five major world religions about frequently encountered medical situations at the end of life (EoL).
This was a systematic review of observational studies on the religious aspects of commonly encountered EoL situations. The databases used for retrieving studies were: Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, Ovid PsycINFO, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, and Scopus. Observational studies, including surveys from healthcare providers or the general population, and case studies were included for review. Articles written from a purely theoretical or philosophical perspective were excluded.
Our search strategy generated 968 references, 40 of which were included for review, while 5 studies were added from reference lists. Whenever possible, we organized the results into five categories that would be clinically meaningful for palliative care practices at the EoL: advanced directives, euthanasia and physician-assisted suicide, physical requirements (artificial nutrition, hydration, and pain management), autopsy practices, and other EoL religious considerations. A wide degree of heterogeneity was observed within religions, depending on the country of origin, level of education, and degree of intrinsic religiosity.
Significance of results:
Our review describes the religious practices pertaining to major EoL issues and explains the variations in EoL decision making by clinicians and patients based on their religious teachings and beliefs. Prospective studies with validated tools for religiosity should be performed in the future to assess the impact of religion on EoL care.
Quasi-one-dimensional (quasi-1D) tools developed for capturing flow and acoustic dynamics in non-segmented solid rocket motors are evaluated using multi-dimensional computational fluid dynamic simulations and used to characterise damping of modal perturbations. For motors with high length-to-diameter ratios (of the order of 10), remarkably accurate estimates of frequencies and damping rates of lower modes can be obtained using the the quasi-1D approximation. Various grain configurations are considered to study the effect of internal geometry on damping rates. Analysis shows that lower cross-sectional area at the nozzle entry plane is found to increase damping rates of all the modes. The flow-turning loss for a mode increases if the more mass addition due to combustion is added at pressure nodes. For the fundamental mode, this loss is, therefore, maximum if burning area is maximum at the centre. The insights from this study in addition to recommendations made by Blomshield(1) based on combustion considerations would be very helpful in realizing rocket motors free from combustion instability.
The spectral classifications of the stars from spectral data have been corrected from time to time and new spectral and luminosity classes have been assigned. Identifying stars with wrong spectral and luminosity classification has been a stupendous task from the huge catalogue of stars. In this work we describe a simple statistical technique to identify stars with wrong spectral and luminosity classification. We make use of the Hipparcos catalogue which has the most accurate measurement of the distance d of the stars. A comparison is made between the absolute V magnitudes MV computed using the observed V magnitude mV and d, with the standard absolute magnitude MV0 assigned to a spectral and luminosity classification for a large number of stars (with d < 100 pc). As expected, for most of the stars the difference between MV and MV0 lies within the range ±2 mag, due to the intrinsic nature of each star ignored in this generalisation. A systematic error analysis is made of all the observable used in the computation. Therefore to identify stars which we suspect to be wrongly classified, we look for abnormal deviation in |MV – MV0| ≥5. The location of these stars with respect to the galactic plain and interstellar extinction is also investigated to rule out effects due to variations in the interstellar extinction. From our results we see that some of the stars were indeed wrongly classified and have recently been reclassified (SIMBAD). The reclassification drastically reduces the |MV – MV0| deviation. The other stars in the list which have not yet been reclassified need to be spectroscopically investigated and classified again.
The West Virginia University Hot hELIcon eXperiment (HELIX) provides variable density and ion temperature plasmas, with controllable levels of thermal anisotropy, for space relevant laboratory experiments in the Large Experiment on Instabilities and Anisotropy (LEIA) as well as fundamental studies of helicon source physics in HELIX. Through auxiliary ion heating, the ion temperature anisotropy (T⊥/T∥) is variable from 1 to 20 for parallel plasma beta (β = 8πnkTi∥/B2) values that span the range of 0.0001 to 0.01 in LEIA. The ion velocity distribution function is measured throughout the discharge volume in steady-state and pulsed plasmas with laser induced fluorescence (LIF). The wavelengths of very short wavelength electrostatic fluctuations are measured with a coherent microwave scattering system. Operating at low neutral pressures triggers spontaneous formation of a current-free electric double layer. Ion acceleration through the double layer is detected through LIF. LIF-based velocity space tomography of the accelerated beam provides a two-dimensional mapping of the bulk and beam ion distribution functions. The driving frequency for the m = 1 helical antenna is continuously variable from 8.5 to 16 MHz and frequency dependent variations of the RF coupling to the plasma allow the spontaneously appearing double layers to be turned on and off without modifying the plasma collisionality or magnetic field geometry. Single and multi-species plasmas are created with argon, helium, nitrogen, krypton, and xenon. The noble gas plasmas have steep neutral density gradients, with ionization levels reaching 100% in the core of the plasma source. The large plasma density in the source enables the study of Aflvén waves in the HELIX device.
Socio-behavioural factors and pathogens associated with childhood diarrhoea are of global public health concern. Our survey in 696 children aged ⩽2 years in rural West Bengal detected rotavirus as sole pathogen in 8% (17/199) of diarrhoeic stool specimens. Other organisms were detected along with rotavirus in 11% of faecal specimens. A third of the children with rotavirus diarrhoea, according to Vesikari score, had severe illness. The top four rotavirus genotypes were G9P (28%), G1P (19%), G2P (14%) and G8P (8%). In the multivariate model, the practice of ‘drawing drinking water by dipping a pot in the storage vessel’ [adjusted odds ratio (aOR) 2·21, 95% confidence interval (CI) 1·03–4·74, P = 0·041], and ‘children aged ⩽6 months with non-exclusive breastfeeding’ (aOR 2·07, 95% CI 1·1–3·82, P = 0·024) had twice the odds of having diarrhoea. Incidence of rotavirus diarrhoea was 24/100 child-years in children aged >6–18 months, 19/100 child-years in children aged >18–24 months and 5/100 child-years in those aged ⩽6 months. Results have translational implications for future interventions including vaccine development.
Diarrhoeal management practices are unsatisfactory in India especially in the slum areas. Dearth of information regarding physicians' diarrhoea-related knowledge and practice in India ncessitated this cross-sectional study of allopathic practitioners in the slums of Kolkata, to assess the distribution and interrelationship between physicians' characteristics, knowledge and practice regarding diarrhoea. A total of 264 randomly selected consenting practitioners were interviewed using a field-tested questionnaire. Nineteen percent had good overall knowledge, 49% and 80% prescribed antibiotics to diarrhoea and cholera patients, respectively, and 55% advised stool examination for every case. Qualified and Government physicians had better knowledge regarding diarrhoea [MBBS: odds ratio (OR) 5·96, P < 0·001; postgraduates: OR 9·33, P < 0·001; Government physicians: OR 11·49, P < 0·0001] and were less likely to prescribe antibiotics for all diarrhoea cases (MBBS: OR 0·30, P = 0·002; postgraduates: OR 0·20, P < 0·001; Government physicians OR 0·24, P < 0·029). Better knowledge was associated with a lower likelihood of prescribing antibiotics for diarrhoea (OR 0·72, P < 0·001), cholera (OR 0·78, P = 0·027) and investigative procedure (OR 0·85, P = 0·028). In the slums of Kolkata, diarrhoea-related knowledge and practice were poor with the exception of qualified physicians, hence an improvement in the knowledge of pharmacists and unqualified practitioners is necessary for the overall improvement of diarrhoeal management in these slums.
Human ability to manipulate atoms and molecules on quantum basis has generated a new dimension of physical structures for molecular scale transistors and devices. We will discuss about nanodimensional single electron transistor. This molecular device works as a switching element by controlling the electron tunneling for amplifying the current. The basic structure consists of two tunnel junctions isolated by a common insulator of nanodimensional length.
One broader aspect of nano power electronics is that, it has got significant role in nanodimensional device regime as tunneling diodes. They have got inherently fast tunneling rate, which makes them highly suitable for high-speed operation. A special type of tunneling diode is an interband tunneling diode (ITD), which is actually, a p-n diode. The V-I characteristics of such diodes are dependent upon the tunneling barrier and tunneling process itself. Another special feature of these diodes is their negative-differential-resistance characteristics. This special characteristic of such diodes makes them very useful in switching digital circuits.
Recently, a new hydrogen NMR signal has been observed in a number of PECVD prepared hydrogenated amorphous silicon (a-Si:H) films of varying quality. It is speculated that the signal is the consequence of a dipolar-coupled hydrogen pair separated, on average, by 1.8 ± 0.1 Å. To elucidate the possible bonding configurations responsible for the NMR data of ref. , we have used ab initio simulation methods to determine a set of relaxed structures of a-Si:H with varying void sizes and H-concentrations. Models containing two isolated hydrogen atoms indicate a preferred H-H distance of approximately 1.8 Å when the two atoms bond to nearest neighbor silicon atoms. This separation also occurs for models containing small, hydrogenated voids, but the configurations giving rise to this H-H distance do not appear to be unique. For larger voids, a proton separation of about 2.4Å is seen, as noted previously . There appears to be consistency between the computed structures and the NMR data for configurations consisting of isolated hydrogen pairs or for clusters of an even number of hydrogen atoms with the constraint that the average H-H distance is 1.8 Å. In this paper, we will discuss the most probable bonding configurations of clustered hydrogen based upon the extent of the NMR data and simulated structures.
While the cause of Parkinson's disease (PD) remains unknown, evidence suggests certain environmental factors, such as well water drinking, herbicides, pesticides exposure and neurotoxins, may trigger the chain of oxidative reactions culminating in the death of dopaminergic neurons in substantia nigra to cause Parkinsonism. To investigate the possible impact of environmental risk factors for idiopathic PD, a case-control study was performed in the Eastern India.
During the period from January 1st, 2006 and December 10th, 2009, 175 PD patients (140 men, 35 women) and 350 non-Parkinson age-sex matched controls were included in the study. Subjects were given a structured neurological examination and completed an administered questionnaire which elicited detailed information on demographic data, pesticides, herbicides family history, occupation, dietary and smoking habits.
The multivariate analysis revealed that family history of PD, pesticide exposure, exposure to toxins other than pesticides and herbicides, rural living and previous history of depression were associated with increased risk of PD, whereas, smoking appeared to be a protective factor. Well water drinking for at least five years, though a significant risk factor on univariate analysis (OR=4.5, 95% CI=2.1-9.9), could not be proved significant in multivariate analysis. Head trauma, vegetarian dietary habit, occupation involving physical exertion and exposure to domestic pets were not as significant risk factors.
Results of our study support the hypothesis of multifactorial etiology of PD with environmental factors acting on a genetically susceptible host.
Objectives and Methods: Many authors have argued that ethical, legal, and social issues (“ELSIs”) should be explicitly integrated into health technology assessment (HTA), yet doing so poses challenges. This discussion may be particularly salient for technologies viewed as ethically complex, such as genetic screening. Here we provide a brief overview of contemporary discussions of the issues from the HTA literature. We then describe key existing policy evaluation frameworks in the fields of disease screening and public health genomics. Finally, we map the insights from the HTA literature to the policy evaluation frameworks, with discussion of the implications for HTA in genetic screening.
Results and Conclusions: A critical discussion in the HTA literature considers the definition of ELSIs in HTA, highlighting the importance of thinking beyond ELSIs as impacts of technology. Existing HTA guidance on integrating ELSIs relates to three broad approaches: literature synthesis, involvement of experts, and consideration of stakeholder values. The thirteen key policy evaluation frameworks relating to disease screening and public health genomics identified a range of ELSIs relevant to genetic screening. Beyond straightforward impacts of screening, these ELSIs require consideration of factors such as the social and political context surrounding policy decisions. The three broad approaches to addressing ELSIs described above are apparent in the screening/genomics literatures. In integrating these findings we suggest that the method chosen for addressing ELSIs in HTA for genetic screening may determine which ELSIs are prioritized; and that an important challenge is the lack of guidance for evaluating such methods.
The starting process in a supersonic nozzle is numerically simulated. The Navier Stokes equations, in axisymmetric form, are solved using a higher order spatial and temporal accurate scheme. Good comparisons between experimental and numerical values of various flow parameters form the basis of further analysis. The insight of the starting process in the nozzle, namely, the movement of primary and secondary shocks and contact discontinuity, has been obtained through analysis of various flow parameters. It has been observed that the inviscid phenomenon is more predominant in the flow development process. Parametric studies have been carried out to determine the effect of nozzle divergence angle on the starting process.
Horsegram (Macrotyloma uniflorum) is sown late in the rainy season by resource-poor farmers in marginal, drought-prone areas of India. Sowing and early crop growth coincide with declining rainfall so crop establishment is often poor and yields are low. Horsegram is a ‘neglected’ crop and farmers' choice of varieties to grow is limited to poor-yielding landraces or to modern varieties for which access to seed is limited. In on-station trials, we compared the yield and relative merits over three years of seven varieties potentially suited to conditions in Jharkhand State and sown with or without priming by soaking seeds in water prior to sowing. Yield stability of the varieties was tested using data from these and five additional trials. Farmers' opinions were recorded during six participatory exercises based on farmers' trials, and their rankings were compared with those from the on-station evaluations. Significant variety × year interactions were observed and yield was generally inversely proportional to drought. Both linear and non-linear genotype × environment interactions were significant for all varieties. Variety BK 1 was the most desirable with stable yields across environments and a higher overall mean grain yield. Variety VLG 1 was unresponsive to more favourable environments but yielded more in the low yielding environments. However, it was the earliest to mature and was identified as a promising variety, which farmers preferred for its grain and brown seeds. This could be used to diversify farming systems with additional options for farmers. Although soaking seeds for 12 h was most effective in increasing germination in an in vitro study, soaking for 8 h before sowing increased emergence and final stand by 11 % and grain yield by 10 % averaged over all varieties and years. Soaking advanced flowering by 1.3 d and maturity by 1.9 d. All varieties responded positively to soaking and did so in all three years, although the response was stronger in drier years.
A maize-breeding programme was targeted at resource-poor farmers of eastern India using a client-oriented approach (often called participatory plant breeding). Farmers tested a range of varieties in a participatory varietal selection programme but none proved to be very popular. Information from these trials was used in a breeding programme to develop a broadly-based population from three yellow- and three white-grained maize varieties that were either identified in the trials or had traits liked by farmers. The population was subjected to several cycles of random mating. In the C3, C4 and C6 cycles, subpopulations were extracted and subjected to mass selection over several generations in environments that represented well the target population of environments of resource-poor farmers' fields in eastern India for traits identified by farmers. To identify better the required traits, in some generations the selection was carried out by farmers on a research farm under fertility levels that approximated farmers' practice. The improvement of the subpopulations resulted in several varieties that performed well in research station and on-farm trials. One of them, BVM 2, was released in Jharkhand state, India. In multilocational research station trials, it yielded more than the control variety BM 1 but silked earlier. In the less favourable environments of on-farm trials, its yield superiority, in percentage terms, was higher. Farmers perceived BVM 2 to have better grain quality and stover yield than the local varieties. BVM 2 was specifically bred to meet the needs of the clients (resource-poor farmers with no access to irrigation) and has earlier maturity combined with higher grain yield. The returns were higher from this highly client-oriented approach, than by classical breeding, mainly because uptake was faster as a result of research and extension being done in tandem.
The low-cristobalite-type modification of Al0.5Ga0.5PO4 is prepared by annealing the amorphous precipitate of stoichiometric phosphate at 1300 °C. The phase purity of the sample is ascertained by powder X-ray diffraction. The crystal structure is refined by Rietveld refinements of the neutron and X-ray diffraction data of the polycrystalline powder. This compound crystallizes in an orthorhombic lattice with unit cell parameters, a=7.0295(8), b=7.0132(8), and c=6.9187(4) Å, V=341.08(6) Å3, Z=4 (Space group C 2221, No. 20). The crystal structure analysis reveals the random distribution of the Al3+ and Ga3+ having tetrahedral coordination with typical M–O (M=Al3+:Ga3+) bond lengths as 1.74 Å. Similarly, the P5+ have tetrahedral coordination with typical P–O bond lengths 1.52–1.54 Å. The Mo4 and PO4 tetraheda are linked by common corners forming a three-dimensional framework lattice. The details of the crystal structure are presented in this paper.