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Congenital heart disease (CHD) is the most common birth defect for infants born in the United States, with approximately 36,000 affected infants born annually. While mortality rates for children with CHD have significantly declined, there is a growing population of individuals with CHD living into adulthood prompting the need to optimise long-term development and quality of life. For infants with CHD, pre- and post-surgery, there is an increased risk of developmental challenges and feeding difficulties. Feeding challenges carry profound implications for the quality of life for individuals with CHD and their families as they impact short- and long-term neurodevelopment related to growth and nutrition, sensory regulation, and social-emotional bonding with parents and other caregivers. Oral feeding challenges in children with CHD are often the result of medical complications, delayed transition to oral feeding, reduced stamina, oral feeding refusal, developmental delay, and consequences of the overwhelming intensive care unit (ICU) environment. This article aims to characterise the disruptions in feeding development for infants with CHD and describe neurodevelopmental factors that may contribute to short- and long-term oral feeding difficulties.
Differential susceptibility theory (DST) posits that individuals differ in their developmental plasticity: some children are highly responsive to both environmental adversity and support, while others are less affected. According to this theory, “plasticity” genes that confer risk for psychopathology in adverse environments may promote superior functioning in supportive environments. We tested DST using a broad measure of child genetic liability (based on birth parent psychopathology), adoptive home environmental variables (e.g., marital warmth, parenting stress, and internalizing symptoms), and measures of child externalizing problems (n = 337) and social competence (n = 330) in 54-month-old adopted children from the Early Growth and Development Study. This adoption design is useful for examining DST because children are placed at birth or shortly thereafter with nongenetically related adoptive parents, naturally disentangling heritable and postnatal environmental effects. We conducted a series of multivariable regression analyses that included Gene × Environment interaction terms and found little evidence of DST; rather, interactions varied depending on the environmental factor of interest, in both significance and shape. Our mixed findings suggest further investigation of DST is warranted before tailoring screening and intervention recommendations to children based on their genetic liability or “sensitivity.”
Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population.
The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials.
The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner.
Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.
Drive-through clinics (DTCs) are a novel type of point of dispensing where participants drive to a designated location and receive prophylaxis while remaining inside their vehicle. The objective of this review was to identify effective practices and recommendations for implementing DTCs for mass prophylaxis dispensing during emergency events.
A systematic review was conducted for articles covering DTCs published between 1990 and 2019. Inclusion criteria were peer-reviewed, written in English, and addressed DTCs sufficiently. Effective practices and recommendations identified in the literature were presented by theme.
A total of 13 articles met inclusion criteria. The themes identified were (1) optimal DTC design and planning via decision support systems and decision support tools; (2) clinic layouts, locations, and design aspects; (3) staffing, training, and DTC communication; (4) throughput time; (5) community outreach methods; (6) DTC equipment; (7) infection prevention and personal protective equipment; and (8) adverse events prevention and traffic management.
DTCs are an essential component of emergency preparedness and must be optimally designed and implemented to successfully dispense mass prophylaxis to a community within 48 hours. The effective practices and recommendations presented can be used for the development, implementation, and improvement of DTCs for their target populations.
We report on the successful demonstration of a 150 J nanosecond pulsed cryogenic gas cooled, diode-pumped multi-slab Yb:YAG laser operating at 1 Hz. To the best of our knowledge, this is the highest energy ever recorded for a diode-pumped laser system.
In this paper we review the design and development of a 100 J, 10 Hz nanosecond pulsed laser, codenamed DiPOLE100X, being built at the Central Laser Facility (CLF). This 1 kW average power diode-pumped solid-state laser (DPSSL) is based on a master oscillator power amplifier (MOPA) design, which includes two cryogenic gas cooled amplifier stages based on DiPOLE multi-slab ceramic Yb:YAG amplifier technology developed at the CLF. The laser will produce pulses between 2 and 15 ns in duration with precise, arbitrarily selectable shapes, at pulse repetition rates up to 10 Hz, allowing real-time shape optimization for compression experiments. Once completed, the laser will be delivered to the European X-ray Free Electron Laser (XFEL) facility in Germany as a UK-funded contribution in kind, where it will be used to study extreme states of matter at the High Energy Density (HED) instrument.
Population-based prevalence and incidence studies are essential for understanding the burden of frontotemporal dementia (FTD).
The MEDLINE and EMBASE databases were searched to identify population-based publications from 1985 to 2012, addressing the incidence and/or prevalence of FTD. References of included articles and prior systematic reviews were searched for additional studies. Two reviewers screened all abstracts and full-text reviews, abstracted data and performed quality assessments.
Twenty-six studies were included. Methodological limitations led to wide ranges in the estimates for prevalence (point prevalence 0.01-4.6 per 1000 persons; period prevalence 0.16-31.04 per 1000 persons) and incidence (0.0-0.3 per 1000 person-years). FTD accounted for an average of 2.7% (range 0-9.1%) of all dementia cases among prevalence studies that included subjects 65 and older compared to 10.2% (range 2.8-15.7%) in studies restricted to those aged less than 65. The cumulative numbers of male (373 [52.5%]) and female (338 [47.5%]) cases from studies reporting this information were nearly equal (p=0.18). The behavioural variant FTD (bvFTD) was almost four times as common as the primary progressive aphasias.
Population-based estimates for the epidemiology of FTD varied widely in the included studies. Refinements in the diagnostic process, possibly by the use of validated biomarkers or limiting case ascertainment to specialty services, are needed to obtain more precise estimates of the prevalence and incidence of FTD.
Population-based prevalence and incidence studies are essential for understanding the societal burden of dementia with Lewy bodies (DLB).
The MEDLINE and EMBASE databases were searched to identify publications addressing the incidence and/or prevalence of DLB. References of included articles and prior systematic reviews were searched for additional studies. Two reviewers screened all abstracts and full-text reviews, abstracted data and performed quality assessments.
Twenty-two studies were included. Incidence rates ranged from 0.5 to 1.6 per 1000 person-years. DLB accounted for 3.2-7.1% of all dementia cases in the incidence studies. Point and period prevalence estimates ranged from 0.02 to 63.5 per 1000 persons. Increasing prevalence estimates were reported with increasing age. DLB accounted for from 0.3 to 24.4% of all cases of dementia in the prevalence studies.
DLB becomes more common with increasing age and accounts for about 5% of all dementia cases in older populations.
Updated information on the epidemiology of dementia due to Alzheimer’s disease (AD) is needed to ensure that adequate resources are available to meet current and future healthcare needs. We conducted a systematic review and meta-analysis of the incidence and prevalence of AD.
The MEDLINE and EMBASE databases were searched from 1985 to 2012, as well as the reference lists of selected articles. Included articles had to provide an original population-based estimate for the incidence and/or prevalence of AD. Two individuals independently performed abstract and full-text reviews, data extraction and quality assessments. Random-effects models were employed to generate pooled estimates stratified by age, sex, diagnostic criteria, location (i.e., continent) and time (i.e., when the study was done).
Of 16,066 abstracts screened, 707 articles were selected for full-text review. A total of 119 studies met the inclusion criteria. In community settings, the overall point prevalence of dementia due to AD among individuals 60+ was 40.2 per 1000 persons (CI95%: 29.1-55.6), and pooled annual period prevalence was 30.4 per 1000 persons (CI95%: 15.6-59.1). In community settings, the overall pooled annual incidence proportion of dementia due to AD among individuals 60+ was 34.1 per 1000 persons (CI95%: 16.4-70.9), and the incidence rate was 15.8 per 1000 person-years (CI95%: 12.9-19.4). Estimates varied significantly with age, diagnostic criteria used and location (i.e., continent).
The burden of AD dementia is substantial. Significant gaps in our understanding of its epidemiology were identified, even in a high-income country such as Canada. Future studies should assess the impact of using such newer clinical diagnostic criteria for AD dementia such as those of the National Institute on Aging–Alzheimer’s Association and/or incorporate validated biomarkers to confirm the presence of Alzheimer pathology to produce more precise estimates of the global burden of AD.
Dementia is a common neurological condition affecting many older individuals that leads to a loss of independence, diminished quality of life, premature mortality, caregiver burden and high levels of healthcare utilization and cost. This is an updated systematic review and meta-analysis of the worldwide prevalence and incidence of dementia.
The MEDLINE and EMBASE databases were searched for relevant studies published between 2000 (1985 for Canadian papers) and July of 2012. Papers selected for full-text review were included in the systematic review if they provided an original population-based estimate for the incidence and/or prevalence of dementia. The reference lists of included articles were also searched for additional studies. Two individuals independently performed abstract and full-text review, data extraction, and quality assessment of the papers. Random-effects models and/or meta-regression were used to generate pooled estimates by age, sex, setting (i.e., community, institution, both), diagnostic criteria utilized, location (i.e., continent) and year of data collection.
Of 16,066 abstracts screened, 707 articles were selected for full-text review. A total of 160 studies met the inclusion criteria. Among individuals 60 and over residing in the community, the pooled point and annual period prevalence estimates of dementia were 48.62 (CI95%: 41.98-56.32) and 69.07 (CI95%: 52.36-91.11) per 1000 persons, respectively. The respective pooled incidence rate (same age and setting) was 17.18 (CI95%: 13.90-21.23) per 1000 person-years, while the annual incidence proportion was 52.85 (CI95%: 33.08-84.42) per 1,000 persons. Increasing participant age was associated with a higher dementia prevalence and incidence. Annual period prevalence was higher in North America than in South America, Europe and Asia (in order of decreasing period prevalence) and higher in institutional compared to community and combined settings. Sex, diagnostic criteria (except for incidence proportion) and year of data collection were not associated with statistically significant different estimates of prevalence or incidence, though estimates were consistently higher for females than males.
Dementia is a common neurological condition in older individuals. Significant gaps in knowledge about its epidemiology were identified, particularly with regard to the incidence of dementia in low- and middle-income countries. Accurate estimates of prevalence and incidence of dementia are needed to plan for the health and social services that will be required to deal with an aging population.
A survey of nearshore areas in the Vestfold Hills, Antarctica, using high-resolution multibeam swath bathymetry provided both a detailed digital bathymetric model and information on sediment acoustic backscatter. Combined with underwater video transects and sediment sampling, these data were used to identify and map geomorphic units. Six geomorphic units identified in the survey region include: rocky outcrops, basins, pediments, valleys, scarps and embayments. In addition to geomorphic units, the data revealed sedimentary features that provide insights into post-glacial sediment transport and erosion in the area. Ice keel pits and scours are common, and sea floor channels, scour depressions and sand ribbons indicate transport and deposition by wind-driven currents and oceanographic circulation. Gullies and sediment lobes observed on steep slopes indicate mass movement of sediment. Some of these processes have not been directly observed to date, but their effectiveness in shaping the modern sea floor is clearly indicated by the sea floor mapping data. The embayments preserve a mantle of boulder sand probably deposited by cold-based glaciers which were flanked by faster-flowing ice in adjoining regions.
This is an exciting era to be a scientist, due to the increasing levels of technology available to us; particularly due to the advances that have been made in the field of computer science. Advances in electronic technology have made computer hardware very powerful and affordable, while advances in software design and human/computer interfaces have made computers more natural to use. Computers have been utilized in nearly every field of scientific study, permitting new levels of development in those fields that wouldn't be possible otherwise. Computer graphics are useful visualization tools that help scientists understand complex data, and convey ideas to others.
Taphonomic processes in deep-water environments differ markedly from those in shallow waters. These differences are illustrated by the preservational style of a large cetacean skeleton lying at the bottom of the Santa Catalina Basin in 1,240 m of water. The degree of skeletal articulation contrasts with that documented in the shallow North Sea where gas-filled, buoyant carcasses disarticulated during flotation. Increased hydrostatic pressure at greater depth is presumed to have prevented the whale carcass from floating and promoted increased levels of preservation. We present a model that relates gas evolution during decay to carcass buoyancy with depth. Application of this model may ultimately allow the degree of skeletal articulation to be used as a rough index of paleobathymetry.
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