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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.
Androgen deprivation therapy (ADT) is used widely as part of a combined modality for the treatment of prostate cancer. However, ADT has also been associated with the development of cardiometabolic complications that can increase mortality from cardiovascular events. There is emerging evidence to suggest that ADT-related cardiometabolic risk can be mitigated by diet and lifestyle modification. While the clinical focus for a nutritional approach for achieving this effect is unclear, it may depend upon the timely assessment and targeting of dietary changes to the specific risk phenotype of the patient. The present review aims to address the metabolic origins of ADT-related cardiometabolic risk, existing evidence for the effects of dietary intervention in modifying this risk, and the priorities for future dietary strategies.
The advent of statins has revolutionised the treatment of patients with raised plasma cholesterol and increased cardiovascular risk. However, the beneficial effects of this class of drugs are far greater than would be expected from lowering of cholesterol alone, and they appear to offer cardiovascular protection at multiple levels, primarily as a result of their pleiotropic activity. Indeed, their favourable effects on the heart seem to be mediated in part through reduced prenylation and subsequent inhibition of small GTPases, particularly those of the Rho family. Such statin-mediated effects are manifested by reduced onset of heart failure and improvements in cardiac dysfunction and remodelling in heart failure patients. Experimental studies have shown that statins mediate their effects on the two major resident cell types of the heart–cardiomyocytes and cardiac fibroblasts–and thus facilitate improvement of adverse remodelling of ischaemic or non-ischaemic aetiology. This review examines evidence for the cellular effects of statins in the heart, and discusses the underlying molecular mechanisms at the level of the cardiomyocyte (hypertrophy, cell death and contractile function) and the cardiac fibroblast (differentiation, proliferation, migration and extracellular matrix synthesis). The prospects for future therapies and ongoing clinical trials are also summarised.
Three years following the global outbreak of severe acute respiratory syndrome (SARS), a national, Web-based survey of Canadian nurses was conducted to assess perceptions of preparedness for disasters and access to support mechanisms, particularly for nurses in emergency and critical care units.
The following hypotheses were tested: (1) nurses' sense of preparedness for infectious disease outbreaks and naturally occurring disasters will be higher than for chemical, biological, radiological, and nuclear (CBRN)-type disasters associated with terrorist attacks; (2) perceptions of preparedness will vary according to previous outbreak experience; and (3) perceptions of personal preparedness will be related to perceived institutional preparedness.
Nurses from emergency departments and intensive care units across Canada were recruited via flyer mailouts and e-mail notices to complete a 30-minute online survey.
A total of 1,543 nurses completed the survey (90% female; 10% male). The results indicate that nurses feel unprepared to respond to large-scale disasters/attacks. The sense of preparedness varied according to the outbreak/disaster scenario with nurses feeling least prepared to respond to a CBRN event. A variety of socio-demographic factors, notably gender, previous outbreak experience (particularly with SARS), full-time vs. part-time job status, and region of employment also were related to perceptions of risk. Approximately 40% of respondents were unaware if their hospital had an emergency plan for a large-scale outbreak. Nurses reported inadequate access to resources to support disaster response capacity and expressed a low degree of confidence in the preparedness of Canadian healthcare institutions for future outbreaks.
Canadian nurses have indicated that considerably more training and information are needed to enhance preparedness for frontline healthcare workers as important members of the response community.
Human clinical trials have shown that fish oils reduce the risk of a variety of disorders including CVD. Despite this, results have been inconsistent. Fish oils are easily oxidised and some fish oils contain higher than recommended levels of oxidised products, but their effects have not been investigated. Recent evidence indicates that dietary oxidised fats can contribute to the development of atherosclerosis and thrombosis. This review summarises findings from cellular, animal and human trials that have examined the effects of oxidised lipids and their potential to affect health outcomes, and proposes that oxidised products in fish oils may attenuate their beneficial effects. More research is required to determine the magnitude of negative effects of fish oil on health outcomes in clinical trials.
Patterning proteins at the sub-micron and nanoscale has many uses, including fabrication of protein arrays for diagnostic and sensor applications. Many groups have reported micron scale protein patterns using photolithography; however, smaller scales have not been realized. In this study, we demonstrate sub-micron protein patterns using photolithography. Patterning is achieved by chemical transformation of pH-reactive polymer films. Site-specific immobilization of streptavidin within a protein resistant background is demonstrated. This methodology could be utilized for the development of high density proteins arrays for biotechnology applications.
Researchers in different parts of the southwestern United States continue to debate whether the end of the Basketmaker period coincides with a general shift from supplemental to intensive maize agriculture across the U.S. Southwest. In some areas this transition appears to have occurred earlier, with heavy reliance on agriculture appearing by the Basketmaker II period. In this study, evidence from dental caries in southwestern Colorado populations supports the latter view, suggesting that Basketmaker subsistence in this area included a heavy reliance on agricultural products. Dental caries frequencies in both Basketmaker and post-Basketmaker samples are well within the expected range for full-time agriculturalists. Although there is no significant association between time period and caries rate, frequencies of interproximal caries and numbers of carious teeth per individual may indicate maize-processing differences between samples obtained from the two temporal periods. Differences in the intensity of maize production, rather than consumption, may contribute to the current lack of agreement on the timing of Southwestern agricultural dependence.