Estimates of Bowhead Whale (Balaena mysticetus) Numbers in the Beaufort Sea during Late Summer

Broad estimates of bowhead whale numbers in Beaufort Sea outer continental shelf (OCS) waters were calculated based on raw counts of whales seen during aerial surveys conducted in late summer 1982-84, corrected by factors accounting for surface area not sampled, surfaced whales missed by observers, and whales too deep to be seen. Annual estimates ranged from roughly 700-1200 to 3000-3500 whales for the latter half of August and from 2000-2200 to 1600-2900 whales for the first half of September. Additionally, estimates of up to 3000 whales were calculated for subregions of the Beaufort Sea for two separate two-week periods, with adjacent-period estimates of only several hundred whales in the same subregions, implying that whale concentrations were highly transitory. The highest estimate (ca. 3500 whales) accounts for less than half of the estimated 7800 whales in the Bering Sea bowhead population. If the population estimate of 7800 whales is valid, then either a substantial number of whales summered outside Beaufort Sea OCS waters in 1982-84 or bowhead numbers are routinely underestimated by the methods used here, or some combination of both.Key words: bowhead whale, Beaufort Sea, outer continental shelf, abundance estimates, aerial surveysMots clés: baleine franche, mer de Beaufort, au large du plateau continental, évaluations de la quantité, relevés aérien

Bowhead and Gray Whale Distributions, Sighting Rates, and Habitat Associations in the Eastern Chukchi Sea, Summer and Fall 2009–15, with a Retrospective Comparison to 1982–91

We analyzed data from line-transect aerial surveys for marine mammals conducted in the eastern Chukchi Sea (67˚–72˚ N, 157˚–169˚ W) in July to October of 2009–15 to investigate bowhead and gray whale distributions, behaviors, sighting rates, and habitat selection preferences, the last of which allowed direct comparison with results from data collected in this area in 1982–91. Bowhead whales use the eastern Chukchi Sea primarily for migrating between the Beaufort Sea and the Bering Sea, while gray whales use the area to feed on locally abundant benthic amphipods and other prey. Bowhead whales were observed during all survey months and were distributed up to 300 km offshore west and southwest of Point Barrow, Alaska, but without a defined migratory corridor in either summer (July-August) or fall (September-October). Bowhead whale sighting rates (whales per km on effort) were highest in the shelf/trough (51–200 m North) depth zone in the northeastern Chukchi Sea in both summer and fall. This pattern was reflected in habitat selection ratios, which found bowhead whales in summer and fall selecting primarily shelf/trough habitat in the northeastern Chukchi Sea, with shelf habitat (36 – 50 m) being preferred secondarily. Gray whales were observed in all survey months and were distributed primarily within ~95 km of shore between Point Barrow and Icy Cape in the northeastern Chukchi Sea, and about 60–115 km southwest of Point Hope in the southern Chukchi Sea. In both summer and fall, gray whale sighting rates and habitat selection ratios were highest in the shelf/trough (51–200 m South) depth zone in the southern Chukchi Sea. In the northeastern part of the study area, gray whale sighting rates and habitat selection ratios both identified coastal habitat (≤ 35 m) as preferred habitat in summer and shelf/trough (51–200 m North) as preferred habitat in fall. Distribution and habitat associations of bowhead and gray whales remained similar over the 34-year time span with one exception: gray whale preference for shelf/trough habitat in the southern Chukchi Sea is now evident throughout summer and fall, whereas three decades ago gray whale preference for that area was limited to fall only.Nous avons analysé les données provenant de levés aériens par transects linéaires à l’égard de mammifères marins, levés effectués dans l’est de la mer des Tchouktches (67˚–72˚ N, 157˚–169˚ O) d’octobre à juillet 2009 à 2015 afin de prélever des données sur la répartition des baleines boréales et des baleines grises, sur leurs comportements, sur leurs taux d’observation et sur leurs préférences en matière d’habitat. Ce dernier élément nous a permis de faire des comparaisons directes avec les résultats de la collecte de données effectuée dans cette région entre 1982 et 1991. Les baleines boréales se servent principalement de l’est de la mer des Tchouktches pour migrer entre la mer de Beaufort et la mer de Béring, tandis que les baleines grises empruntent cette région pour se nourrir des amphipodes benthiques qui y abondent ainsi que d’autres proies. Des baleines boréales ont été observées pendant tous les mois visés par les levés, et celles-ci étaient réparties sur une distance allant jusqu’à 300 km au large, à l’ouest et au sud-ouest de Point Barrow, en Alaska, sans toutefois emprunter un couloir migratoire particulier à l’été (juillet et août) ou à l’automne (septembre et octobre). Les taux d’observation de baleines boréales (nombre de baleines par km à l’effort) étaient plus élevés dans la zone de profondeur de plateformes et de dépressions (de 51 à 200 m nord) faisant partie du nord-est de la mer des Tchouktches, tant à l’été qu’à l’automne. Cette tendance se reflète dans les rapports de sélection d’habitats, selon lesquels les baleines boréales choisissent principalement, l’été et l’automne, un habitat de plateformes et de dépressions dans le nord-est de la mer des Tchouktches, l’habitat des plateformes (de 36 à 50 m) constituant une préférence secondaire. Des baleines grises ont été observées pendant tous les mois visés par les levés. Elles se répartissaient principalement à l’intérieur de ~95 km de la côte, entre Point Barrow et Icy Cape, dans le nord-est de la mer des Tchouktches, et à environ 60 à 115 km au sud-ouest de Point Hope, dans le sud de la mer des Tchouktches. Tant à l’été qu’à l’automne, les taux d’observation et les rapports de sélection d’habitats des baleines grises étaient à leur point le plus élevé dans la zone de profondeur des plateformes et des dépressions (de 51 à 200 m sud) du sud de la mer des Tchouktches. Dans le secteur nord-est de la région visée par l’étude, les taux d’observation et les ratios de sélection d’habitats des baleines grises ont tous deux permis d’affirmer que l’habitat côtier (≤ 35 m) était l’habitat préféré pendant l’été, et que l’habitat des plateformes et des dépressions (de 51 à 200 m nord) était l’habitat préféré à l’automne. Les associations en matière de répartition et d’habitat des baleines boréales et des baleines grises sont demeurées semblables au cours de la période de 34 ans, à une exception près : la préférence de la baleine grise pour l’habitat des plateformes et des dépressions dans le sud de la mer des Tchouktches est maintenant évidente à l’été et à l’automne, tandis qu’il y a trois décennies, la préférence de la baleine grise pour cet habitat se limitait à l’automne

Bowhead and Beluga Whale Distributions, Sighting Rates, and Habitat Associations in the Western Beaufort Sea in Summer and Fall 2009–16, with Comparison to 1982–91

We analyzed data from line-transect aerial surveys for marine mammals conducted in the western Beaufort Sea (shore to 72˚ N, 140˚–157˚ W) from July to October of 2009–16 to investigate the distribution, behaviors, sighting rates, and habitat use preferences of bowhead and beluga whales. The habitat use data allowed for direct comparison with data collected in the same area from 1982 to 1991. Both species are ice-adapted, migrating through leads in sea ice in spring, and are seasonal inhabitants of the western Beaufort Sea during summer and fall. From 2009 to 2016, bowheads were seen in all survey months, with the highest overall sighting rate (whales per km) in August. Bowhead sighting rates were highest in the whales’ preferred habitats: outer shelf habitat (51–200 m depth) in July and inner shelf-shallow habitat (≤ 20 m depth) in August, September, and October. Beluga whales were also seen in all survey months, with highest overall sighting rate in July. Beluga whales were overwhelmingly associated with continental slope habitat (201–2000 m depth) in all months. Bowhead distribution and depth preferences in summer months of 2009–16 differed from those observed in 1982–91, when bowheads were not seen during limited survey effort in July and preferred outer continental shelf habitat in August. These differences indicate that bowhead whale preference for shallow shelf habitat now occurs earlier in summer than it used to. Beluga distribution and depth preference remained similar between 1982–91 and 2009–16, with strong preference for continental slope during both periods. Differences in sea ice cover habitat association for both species are likely due more to the relative lack of sea ice in recent years compared to the earlier period than to shifts in habitat preference. Habitat partitioning between bowhead and beluga whales in the western Beaufort Sea remained evident except in July, when both species used continental slope habitat. In July – October 2009–16, the distribution, sighting rates, and behavior of both bowheads and belugas in the western Beaufort showed considerable interannual variation, which underscores the importance of annual sampling to accurate records of the complex western Beaufort Sea ecosystem.Nous avons analysé les données découlant de levés aériens de transects linéaires pour mammifères marins, levés effectués dans l’ouest de la mer de Beaufort (de la rive jusqu’à 72˚ N, et de 140˚ jusqu’à 157˚ O) de juillet à octobre 2009 à 2016. Ces levés avaient pour but d’étudier la distribution, les comportements, les taux d’observation ainsi que les préférences d’utilisation de l’habitat des baleines boréales et des bélugas. Les données relatives à l’utilisation de l’habitat ont permis d’établir des comparaisons directes avec les données recueillies dans le même secteur de 1982 à 1991. Ces deux espèces sont adaptées à la glace, migrent par des chenaux formés dans la glace de mer au printemps et sont des habitants saisonniers de l’ouest de la mer de Beaufort pendant l’été et l’automne. Entre 2009 et 2016, des baleines boréales ont été aperçues pendant tous les mois visés par les levés, le taux d’observation général le plus élevé (nombre de baleines par km) ayant été enregistré au mois d’août. Les taux d’observation des baleines boréales étaient les plus élevés dans les habitats préférés de ces baleines, soit l’habitat de la plateforme externe (de 51 m à 200 m de profondeur) en juillet et l’habitat de la plateforme interne peu profonde (≤ 20 m de profondeur) en août, en septembre et en octobre. Des bélugas ont également été aperçus pendant tous les mois visés par les levés, le taux d’observation général le plus élevé ayant été enregistré en juillet. Les bélugas étaient massivement associés à l’habitat de la pente continentale (de 201 m à 2 000 m de profondeur) pendant tous les mois. La distribution et les préférences de profondeur des baleines boréales pendant les mois d’été 2009 à 2016 différaient de celles observées de 1982 à 1991, lorsque les baleines boréales n’ont pas été aperçues dans le cadre des quelques levés qui ont été effectués en juillet et préféraient leur habitat de la plateforme continentale externe en août. Ces différences indiquent que la préférence des baleines boréales pour l’habitat de la plateforme peu profonde se manifeste maintenant plus tôt l’été qu’auparavant. De 1982 à 1991 et de 2009 à 2016, la distribution des bélugas et leur préférence de profondeur sont restées semblables, avec une préférence marquée pour la pente continentale pendant les deux périodes. Pour les deux espèces, les différences sur le plan de l’association de la couverture de glace marine sont vraisemblablement davantage attribuables au manque relatif de glace de mer ces dernières années comparativement à la période précédente plutôt qu’à une variation de la préférence de l’habitat. Dans l’ouest de la mer de Beaufort, la séparation de l’habitat entre les baleines boréales et les bélugas demeurait évidente, sauf en juillet, quand les deux espèces utilisaient l’habitat de la pente continentale. De juillet à octobre 2009 à 2016, la distribution, les taux d’observation et le comportement des baleines boréales et des bélugas dans l’ouest de la mer de Beaufort ont affiché une variation considérable d’une année à l’autre, ce qui fait ressortir l’importance de faire des échantillonnages annuels afin d’obtenir des données précises au sujet de l’écosystème complexe de l’ouest de la mer de Beaufort

Application of a policy framework for the public funding of drugs for rare diseases

BACKGROUND: In many countries, decisions about the public funding of drugs are preferentially based on the results of randomized trials. For truly rare diseases, such trials are not typically available, and approaches by public payers are highly variable. In view of this, a policy framework intended to fairly evaluate these drugs was developed by the Drugs for Rare Diseases Working Group (DRDWG) at the request of the Ontario Public Drug Programs. OBJECTIVE: To report the initial experience of applying a novel evaluation framework to funding applications for drugs for rare diseases. METHODS: Retrospective observational cohort study. MEASURES: Clinical effectiveness, costs, funding recommendations, funding approval. KEY RESULTS: Between March 2008 and February 2013, eight drugs were evaluated using the DRDWG framework. The estimated average annual drug cost per patient ranged from 28,000 to 1,200,000 Canadian dollars (CAD). For five drugs, full evaluations were completed, specific funding recommendations were made by the DRDWG, and funding was approved after risk-sharing agreements with the manufacturers were negotiated. For two drugs, the disease indications were determined to be ineligible for consideration. For one drug, there was insufficient natural history data for the disease to provide a basis for recommendation. For the five drugs fully evaluated, 32 patients met the predefined eligibility criteria for funding, and five were denied based on predefined exclusion criteria. CONCLUSIONS: The framework improved transparency and consistency for evaluation and public funding of drugs for rare diseases in Ontario. The evaluation process will continue to be iteratively refined as feedback on actual versus expected clinical and economic outcomes is incorporated. © 2014 Society of General Internal Medicine

Beluga whales in the western Beaufort Sea : current state of knowledge on timing, distribution, habitat use and environmental drivers

ECG was supported by a National Research Council-National Academy of Sciences Postdoctoral Fellowship.The seasonal and geographic patterns in the distribution, residency, and density of two populations (Chukchi and Beaufort) of beluga whales (Delphinapterus leucas) were examined using data from aerial surveys, passive acoustic recordings, and satellite telemetry to better understand this arctic species in the oceanographically complex and changing western Beaufort Sea. An aerial survey data-based model of beluga density highlights the Beaufort Sea slope as important habitat for belugas, with westerly regions becoming more important as summer progresses into fall. The Barrow Canyon region always had the highest relative densities of belugas from July-October. Passive acoustic data showed that beluga whales occupied the Beaufort slope and Beaufort Sea from early April until early November and passed each hydrophone location in three broad pulses during this time. These pulses likely represent the migrations of the two beluga populations: the first pulse in spring being from Beaufort animals, the second spring pulse Chukchi belugas, with the third, fall pulse a combination of both populations. Core-use and home range analyses of satellite-tagged belugas showed similar use of habitats as the aerial survey data, but also showed that it is predominantly the Chukchi population of belugas that uses the western Beaufort, with the exception of September when both populations overlap. Finally, an examination of these beluga datasets in the context of wind-driven changes in the local currents and water masses suggests that belugas are highly capable of adapting to oceanographic changes that may drive the distribution of their prey.PostprintPeer reviewe

Childhood onset of Scheie syndrome, the attenuated form of mucopolysaccharidosis I

Scheie syndrome is the most attenuated and rarest form of mucopolysaccharidosis type I (MPS I), an inherited lysosomal storage disorder. Only small patient series have previously been reported. Using natural history data from the uniquely large population of 78 Scheie patients enrolled in the MPS I Registry, we characterized the onset and prevalence of clinical manifestations and explored reasons for delayed diagnosis of the disease. Median patient age was 17.5 years; 46% of the patients were male, and 88% were Caucasian. Of 25 MPS I-related clinical features, cardiac valve abnormalities, joint contractures, and corneal clouding were each reported by >80% and all three by 53% of patients. Carpal tunnel syndrome, hernia, coarse facial features, and hepatomegaly were each reported by >50% of patients. Age at onset of the clinical features varied widely between individuals, but the median age at onset was 3 years for hernia and between 5 and 12 years for most features, including coarse facial features, hepatomegaly, joint contractures, bone deformities, cardiac valve abnormalities, cognitive impairment, and corneal clouding. Carpal tunnel syndrome, cardiomyopathy, and myelopathy arose more commonly during adolescence or adulthood. Delays up to 47 years intervened between symptom onset and disease diagnosis, and the longest delays were associated with later age at symptom onset and symptom onset before 1980. In summary, Scheie syndrome usually emerges during childhood, and recognition of attenuated MPS I requires awareness of the multisystemic disease manifestations and their diverse presentation. Given the availability of etiologic treatment, prompt diagnosis is important

Prevalence of childhood disability and the characteristics and circumstances of disabled children in the UK : secondary analysis of the Family Resources Survey

Background: Robust data on the prevalence of childhood disability and the circumstances and characteristics of disabled children is crucial to understanding the relationship between impairment and social disadvantage. It is also crucial for public policy development aimed at reducing the prevalence of childhood disability and providing appropriate and timely service provision. This paper reports prevalence rates for childhood disability in the United Kingdom (UK) and describes the social and household circumstances of disabled children, comparing these where appropriate to those of non-disabled children. Methods: Data were generated from secondary analysis of the Family Resources Survey, a national UK cross-sectional survey, (2004/5) which had data on 16,012 children aged 0-18 years. Children were defined as disabled if they met the Disability Discrimination Act (DDA) definition (1995 and 2005). Frequency distributions and cross-tabulations were run to establish prevalence estimates, and describe the circumstances of disabled children. To establish the association between individual social and material factors and childhood disability when other factors were controlled for, logistic regression models were fitted on the dependent variable 'DDA defined disability'. Results: 7.3% (CI 6.9, 7.7) of UK children were reported by as disabled according to the DDA definition. Patterns of disability differed between sexes with boys having a higher rate overall and more likely than girls to experience difficulties with physical coordination; memory, concentration and learning; communication. Disabled children lived in different personal situations from their non-disabled counterparts, and were more likely to live with low-income, deprivation, debt and poor housing. This was particularly the case for disabled children from black/minority ethnic/ mixed parentage groups and lone-parent households. Childhood disability was associated with lone parenthood and parental disability and these associations persisted when social disadvantage was controlled for. Conclusion: These analyses suggest that UK disabled children experience higher levels of poverty and personal and social disadvantage than other children. Further research is required to establish accurate prevalence estimates of childhood disability among different black and minority ethnic groups and to understand the associations between childhood disability and lone parenthood and the higher rates of sibling and parental disability in households with disabled children

Study protocol: a cluster randomised controlled trial of a school based fruit and vegetable intervention – Project Tomato

Background The School Fruit and Vegetable Scheme (SFVS) is an important public health intervention. The aim of this scheme is to provide a free piece of fruit and/or vegetable every day for children in Reception to Year 2. When children are no longer eligible for the scheme (from Year 3) their overall fruit and vegetable consumption decreases back to baseline levels. This proposed study aims to design a flexible multi-component intervention for schools to support the maintenance of fruit and vegetable consumption for Year 3 children who are no longer eligible for the scheme. Method This study is a cluster randomised controlled trial of Year 2 classes from 54 primary schools across England. The schools will be randomly allocated into two groups to receive either an active intervention called Project Tomato, to support maintenance of fruit intake in Year 3 children, or a less active intervention (control group), consisting of a 5 A DAY booklet. Children's diets will be analysed using the Child And Diet Evaluation Tool (CADET), and height and weight measurements collected, at baseline (Year 2) and 18 month follow-up (Year 4). The primary outcome will be the ability of the intervention (Project Tomato) to maintain consumption of fruit and vegetable portions compared to the control group. Discussion A positive result will identify how fruit and vegetable consumption can be maintained in young children, and will be useful for policies supporting the SFVS. A negative result would be used to inform the research agenda and contribute to redefining future strategies for increasing children's fruit and vegetable consumption

Uncovering treatment burden as a key concept for stroke care: a systematic review of qualitative research

<b>Background</b> Patients with chronic disease may experience complicated management plans requiring significant personal investment. This has been termed ‘treatment burden’ and has been associated with unfavourable outcomes. The aim of this systematic review is to examine the qualitative literature on treatment burden in stroke from the patient perspective.<p></p> <b>Methods and findings</b> The search strategy centred on: stroke, treatment burden, patient experience, and qualitative methods. We searched: Scopus, CINAHL, Embase, Medline, and PsycINFO. We tracked references, footnotes, and citations. Restrictions included: English language, date of publication January 2000 until February 2013. Two reviewers independently carried out the following: paper screening, data extraction, and data analysis. Data were analysed using framework synthesis, as informed by Normalization Process Theory. Sixty-nine papers were included. Treatment burden includes: (1) making sense of stroke management and planning care, (2) interacting with others, (3) enacting management strategies, and (4) reflecting on management. Health care is fragmented, with poor communication between patient and health care providers. Patients report inadequate information provision. Inpatient care is unsatisfactory, with a perceived lack of empathy from professionals and a shortage of stimulating activities on the ward. Discharge services are poorly coordinated, and accessing health and social care in the community is difficult. The study has potential limitations because it was restricted to studies published in English only and data from low-income countries were scarce.<p></p> <b>Conclusions</b> Stroke management is extremely demanding for patients, and treatment burden is influenced by micro and macro organisation of health services. Knowledge deficits mean patients are ill equipped to organise their care and develop coping strategies, making adherence less likely. There is a need to transform the approach to care provision so that services are configured to prioritise patient needs rather than those of health care systems

Cancer Treatment and Bone Health

Considerable advances in oncology over recent decades have led to improved survival, while raising concerns about long-term consequences of anticancer treatments. In patients with breast or prostate malignancies, bone health is a major issue due to the high risk of bone metastases and the frequent prolonged use of hormone therapies that alter physiological bone turnover, leading to increased fracture risk. Thus, the onset of cancer treatment-induced bone loss (CTIBL) should be considered by clinicians and recent guidelines should be routinely applied to these patients. In particular, baseline and periodic follow-up evaluations of bone health parameters enable the identification of patients at high risk of osteoporosis and fractures, which can be prevented by the use of bone-targeting agents (BTAs), calcium and vitamin D supplementation and modifications of lifestyle. This review will focus upon the pathophysiology of breast and prostate cancer treatment-induced bone loss and the most recent evidence about effective preventive and therapeutic strategies