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Background: The late-onset cerebellar ataxias (LOCAs) have until recently resisted molecular diagnosis. Contributing to this diagnostic gap is that non-coding structural variations, such as repeat expansions, are not fully accessible to standard short-read sequencing analysis. Methods: We combined bioinformatics analysis of whole-genome sequencing and long-read sequencing to search for repeat expansions in patients with LOCA. We enrolled 66 French-Canadian, 228 German, 20 Australian and 31 Indian patients. Pathogenic mechanisms were studied in post-mortem cerebellum and induced pluripotent stem cell (iPSC)-derived motor neurons from 2 patients. Results: We identified 128 patients who carried an autosomal dominant GAA repeat expansion in the first intron of the FGF14 gene. The expansion was present in 61%, 18%, 15% and 10% of patients in the French-Canadian, German, Australian and Indian cohorts, respectively. The pathogenic threshold was determined to be (GAA)≥250, although incomplete penetrance was observed in the (GAA)250-300 range. Patients developed a slowly progressive cerebellar syndrome at an average age of 59 years. Patient-derived post-mortem cerebellum and induced motor neurons both showed reduction in FGF14 RNA and protein expression compared to controls. Conclusions: This intronic, dominantly inherited GAA repeat expansion in FGF14 represents one of the most common genetic causes of LOCA uncovered to date.
Some slug species are considered a nuisance in agriculture and horticulture worldwide, causing economic losses to growers. Phasmarhabditis is a genus of bacteria-feeding nematodes that can parasitize slugs and snails and thus potentially serve as a biological control agent. Canada had no record of Phasmarhabditis until a survey conducted in 2019 reported a Canadian strain of Phasmarhabditis californica from a single Arion rufus slug. To build on this discovery, we surveyed three major agricultural sites, ten greenhouses, and nurseries in Alberta from June to September 2021 to collect pest slug species and investigate their associated nematodes, specifically P. californica. Slugs were collected from the field and returned to the laboratory to check for emerging nematodes on White traps. We collected 1331 slugs belonging to nine species, with Deroceras reticulatum being the most common. Only 45 (3.38%) slug samples were positive for nematodes, and the majority were identified to species level: Alloionema appendiculatum, Caenorhabditis briggsae, Caenorhabditis elegans, Panagrolaimus subelongatus, and Mesorhabditis spiculigera. We did not isolate P. californica from any of the slugs collected from these survey sites, which included the original site where P. californica was discovered. However, four D. reticulatum slugs retrieved from a residential garden sample were infected with P. californica. These findings suggest the possibility of a fragmented distribution of P. californica across Alberta. Future research should focus on extensively surveying agriculture and horticulture sites and residential gardens in different provinces across Canada.
The COVID-19 pandemic has impacted populations internationally, through infections and consequences of infections, and by the countermeasures to prevent the spread of the coronavirus. Concerns exist surrounding the impact of the COVID-19 pandemic on the mental health of youths in the Netherlands. Consequently, we studied quarterly trends and risk factors of suicidal ideation among Dutch youths from September 2021 onwards.
The Network GOR-COVID-19, a research group consisting of different organizations, monitors the effect of the COVID-19 pandemic on population health. As one element of this monitoring, quarterly data collections have been undertaken since September 2021 from a panel of youths (12 – 25 years of age) representative of the Dutch population. Online questionnaires collect data on self-reported health and wellbeing. We analyzed a selection of demographic, social activity, and mental health variables as potential risk factors for self-reported suicidal ideation in the previous three months. We assessed trends, performed longitudinal analyses, and conducted logistic and random forest regressions per quarterly round of data collection. Analyses were weighted for age, sex, educational level, and province where appropriate.
Approximately 4,500 youths participated in each quarterly questionnaire, with some participating more than once. Results showed substantial increases in self-reported suicidal ideation during and immediately after the third lockdown in the Netherlands in December 2021, rising from 9% to 17%, then slowly decreasing to 16% in May/June 2022, and to 13% in September 2022. In all multivariable analyses variables relating to mental health were indicated as risk factors. The strongest associations were seen in those experiencing mental health complaints and loneliness. Demographic variables were not strongly associated with suicidal ideation.
The monitoring conducted by The Network GOR-COVID-19 enables the tracking of trends in the impact of the COVID-19 pandemic on the mental health of Dutch youths in The Netherlands.
More than 7.8 million people fled Ukraine since the invasion of Russia and are registered as refugees in Europe (as of November 1, 2022). Almost 89,000 of them are registered to the Netherlands (as of November 3, 2022). It is expected that this number will rise. Appropriate and accessible Mental Health and Psychosocial Support (MHPSS) is essential for conflict survivors to address psychological harm from traumatic events and distress both during the escape and after, while trying to adjust to an unfamiliar place. Receiving countries have the obligation to provide MHPSS as part of their international commitment to the right to health. This is recognized in the International Covenant on Economic, Social and Cultural Rights (ICESCR) and the Convention on the Rights of Persons with Disabilities (CRPD). Nevertheless, the Netherlands is failing to honor this commitment with fragmented services that do not seem to fit support needs. The longer it takes to put a comprehensive approach in place, the greater the damage to the refugees will be. This interactive session aims to shed light on practical challenges and opportunities for the implementation of appropriate, accessible and integrated MHPSS. What is needed to go from a fragmented to an integrated approach?
Being active as advisors in the field of Disaster Health and MHPSS in the Netherlands, the presenters review their experienced challenges thereafter opportunities and good practices are explored together with the participants.
Experienced challenges include complexity, fragmented organization, lack of ownership and inadequate access to knowledge and information about support needs.
More is needed to meet the commitment to the right of health and to provide adequate MHPSS to refugees in the Netherlands and beyond. International exchange and learning can help us to understand and overcome implementation challenges.
Despite its common usage, the meaning of ‘democratic’ in democratic intelligence oversight has rarely been spelled out. In this article, we situate questions regarding intelligence oversight within broader debates about the meanings and practices of democracy. We argue that the literature on intelligence oversight has tended to implicitly or explicitly follow liberal and technocratic ideas of democracy, which have limited the understanding of oversight both in academia and in practice. Thus, oversight is mostly understood as an expert, institutional and partially exclusive arrangement that is supposed to strike a balance between individual freedom and collective security, with the goal of establishing the legitimacy of and trust in intelligence work in a national setting. ‘Healthy’ or ‘efficient’ democratic oversight then becomes a matter of technical expertise, non-partisanship, and the ability to guard secrets. By analysing three moments of struggle around what counts as intelligence oversight across Germany, the UK, and the US, this article elucidates their democratic stakes. Through a practice-based approach, we argue that oversight takes much more agonistic, contentious, transnational, and public forms. However, these democratic practices reconfiguring oversight remain contested or contained by dominant views on what constitutes legitimate and effective intelligence oversight.
Science has been invaluable for increasing understanding of animal welfare and as a result we know of many ways in which animal treatment and housing can be improved. However, implementation is slow because of political and economic considerations. This is particularly clear for farm animals. This raises the question of what else is needed, besides science, for implementation of welfare improvements. At least three other disciplines need more attention in this respect: sociology, economics and ethics. Scientists will continue to be central in achieving improvements in animal welfare by providing credible, authoritative information on animal welfare and other issues. But to increase implementation of their results they need to increase dialogue with all of the players involved — producers, retailers, consumers, legislators and the media — as well as with specialists in other disciplines to improve cross-disciplinary understanding.
Chromosome substitution strains (also called consomic lines or strains) are strains in which a single, full-length chromosome from one inbred strain — the donor strain — has been transferred onto the genetic background of a second inbred strain — the host strain. Based on the results obtained from behavioural tests with the two parental strains, the minimum number of animals from each of the host and consomic strains that are required for a successful behavioural genetic analysis can be estimated. Correct application of statistical knowledge can lead to a further reduction in the number of animals used in behavioural genetic experiments using chromosome substitution strains.
The distress experienced by animals during the induction of unconsciousness remains one of the most important and yet overlooked aspects of effective methods of anaesthesia and euthanasia. Here we show that considerable differences exist in the aversive responses elicited by 12 common methods of inhalational anaesthesia and euthanasia in laboratory rats and mice. Carbon dioxide, either alone or in combination with oxygen or argon, was found to be highly aversive to both species. The least aversive agents were halothane in rats and enflurane in mice. Exposing these animals to carbon dioxide in any form, either for anaesthesia or for euthanasia, is likely to cause considerable pain and distress and is therefore unacceptable when efficient and more humane alternatives are readily available.
Dolphins in captivity have to cope with severe changes in their environment. So far, there are few studies on the welfare of these animals under these conditions. The aim of the present study was to find if cortisol was present in the saliva of dolphins and to explore the possibility of performing serial, non-invasive cortisol assays in captive dolphins. Saliva was collected non-invasively during a month from four dolphins that had responded to previous training, in order to provide saliva samples, in two aquaria in Mexico City. In addition, serum and saliva time-matched samples were obtained in an aquarium in Nuevo Vallarta, Mexico. Cortisol concentrations in saliva and blood were measured by radioimmunoanalysis (RIA). Results show for the first time that measurable quantities of cortisol are secreted within the saliva of dolphins. Salivary cortisol measurements could be a useful tool for carrying out long-term cortisol sampling. It is far less invasive than blood-sampling and could be used, in conjunction with behavioural observations, to monitor the welfare of captive dolphins, non-invasively.
Theoretical models are presented of the effects of space, facilities and group size on the behaviour of chickens at high stocking densities, with relevance for all animals. The appropriateness of each model is supported by published data, although such data are scant for some important variables. Freedom of movement is analysed by taking the area of a hen as 475 cm2 and finding the number of free bird spaces left at different space allowances. This provides support for current recommendations of a maximum of seven laying hens per m2 on deep litter, but suggests that a maximum for broilers of 34 kg/m2 unacceptably restricts freedom of movement. In cages, freedom of movement increases with space allowance per hen, and, for a given space allowance, with cage and group size. Nesting behaviour is analysed for synchrony, which decreases with group size. Perching and feeding are often synchronous and the space needed for these is determined by body width. Recommendations are derived for hens in furnished cages. The main part of the cage should be as large as possible; an absolute minimum of 600 cm2 per bird is suggested, but 675 cm2 per bird is probably the minimum practical. Perch and feeder space should be provided at 14 cm or more per bird, with a possible derogation for light hybrids to 12 cm. The number of nest spaces needed varies with the number of birds, with nest spaces being 300 cm2 each. These recommendations sum to a minimum of 800 cm2 per bird for groups of eight or more, 850 cm2 for groups of four to seven, and 900 cm2 for groups of three or fewer, plus litter area. Crowding is primarily caused by limited space allowance, but for a given space allowance it is worse in small enclosures and groups.
The welfare of conventional stock laboratory mice has been assessed in 46 UK animal units using an expert-defined welfare assessment protocol containing 119 measures of mouse welfare. These were recorded using a questionnaire and observations made during a one-day visit to each unit. The standard of mouse welfare was considered to be good with widespread use of substrate and nesting material and space allowances in most cases well above the minimum recommended levels. Education and training was available and encouraged by the majority of animal units. The health and welfare of laboratory mice was being frequently assessed by animal care staff using daily inspections/observations, health records, and health monitoring schemes. Overall the mice assessed could be considered to be in good health, as indicators of poor health and welfare were exhibited at low levels, and the mice were observed exhibiting a wide range of positive natural behaviours. A number of environmental conditions (humidity, noise and light intensity) were outside recommended ranges in some animal units. The provision of cage resources such as shelters, gnawing material, floor food and other enrichment items were found to be variable. A high proportion of the units surveyed housed at least some of their mice (mainly males) singly and handling of mice by care staff varied between units. In some units there may be an opportunity for some staff to improve in some aspects of mouse handling. Finally, a number of interesting correlations were found between various behaviours and potential indictors of abnormal health or welfare, which require further investigation.
The concept of quality of life in animals is closely associated with the concepts of animal sentience and animal welfare. It reflects a positive approach that inquires what animals like or prefer doing. The assessment of farm animal welfare requires a good understanding of the animals' affective experience, including their emotions. However, affective experience in animals is difficult to measure because of the absence of verbal communication. Recent studies in the field of cognitive psychology have shown that affective experience can be investigated without using verbal communication by examination of the interactions between emotions and cognition. On the one hand, appraisal theories provide a conceptual framework which suggests that emotions in humans are triggered by a cognitive process whereby the situation is evaluated on a limited number of elementary criteria such as familiarity and predictability. We have applied these appraisal theories to develop an experimental approach for studying the elementary criteria used by farm animals to evaluate their environment and the combinations of those criteria that trigger emotions. On the other hand, an increasing body of research, first in humans and then in other animals, suggests that emotions also influence cognitive processes by modifying attention, memory and judgement in a short- or long-term manner. Cognitive processes could therefore be manipulated and measured to provide new insights into how not only emotions but also more persistent affective states can be assessed in animals. Further work based on these cognitive approaches will offer new paradigms for improving our understanding of animal welfare, thus contributing to ‘a life of high quality’ in animals.
A Delphi consultation process was used to examine the validity and feasibility of potential resource-input and animal-based outcome measures of laboratory mouse welfare identified by a comprehensive literature search. The consultation was an iterative two-stage technique that used web and email based questionnaires to gather data. On completion of the second consultation stage a total of 97 measures of mouse welfare, their order of importance, and appropriate sample sizes and sampling times where relevant, were identified. Of these, 55 were resource-input measures that included assessments of the cages, animal rooms environmental conditions, husbandry procedures, provision of food and water, staffing, resources placed into mouse cages, and the establishment policies. The remaining 42 were animal-based outcomes, measures that included assessments of unprovoked behaviour, provoked responses, vocalisations, physical appearance, and health indicators. The measures that were considered by the experts to be appropriate for assessing laboratory mouse welfare measures can be placed into four basic categories: characteristics of the staff, performance of husbandry and care procedures, health and well-being of the mice, and the provision and use of cage resources.
This study aimed to identify behaviours that could be used to assess post-operative pain and analgesic efficacy in male rabbits. In consideration of the ‘Three Rs’, behavioural data were collected on seven male New Zealand White rabbits in an ethically approved experiment requiring abdominal implantation of a telemetric device for purposes other than behavioural assessment. Prior to surgery, rabbits were anaesthetised using an isoflurane/oxygen mix and given Carprofen (2 mg kg−1) as a peri-operative analgesic. Rabbits were housed individually in standard laboratory cages throughout. Data were collected at three time periods: 24-21 h prior to surgery (T1) and, post-surgery, 0-3 h (T2) and 3-6 h (T3). Behavioural changes were identified using Observer XT, significance of which was assessed using a Friedman's test for several related samples. The frequency or duration of numerous pre-operative behaviours was significantly reduced in T2 and T3, as compared to T1. Conversely, novel or rare behaviours had either first occurrence or significant increase in T2 into T3 as compared to T1, these include ‘full-body-flexing’, ‘tight-huddling’, ‘hind-leg-shuffling’. We conclude that reduced expression of common pre-operative behaviours and the appearance of certain novel post-operative behaviours may be indicative of pain in rabbits. Behaviours identified as increased in T2 as compared to T1 but not consistently elevated into T3 were considered separately due to the potentially confounding effect of anaesthesia recovery. These included lateral lying, ‘drawing-back’, ‘staggering’ and ‘closed eyes’. We postulate that for effective application of best-practice post-operative care, informed behavioural observation can provide routes by which carers may identify requirements for additional post-operative analgesia. Additionally, improvement of the peri-operative pain management regimen may be required to ameliorate the immediate effects of abdominal surgery. Comparisons with other studies into post-operative pain expression in rabbits suggest behavioural indicators of pain may differ, depending on housing and surgical procedure.
Collar-worn deterrents reduce predation by cats while collar-mounted ID enhances return of lost animals. A perception that collars are hazardous limits their use. We defined cases as ‘collar incidents’ (cat snagged its collar or caught a paw), ‘collar injuries’ (veterinary treatment needed for a collar incident), and ‘collar deaths’ (cat died), before integrating data from veterinarians, owners from the general public and owners from a welfare society. Despite biases associated with each of these groups, taken together, the results from these indicated that collar injuries or deaths are rare. Interviews with one hundred and seven veterinarians indicated an average rate of one collar injury observed per 2.3 years of veterinary practice. At one practice, over three years, only 0.33% of 4,460 cat cases were collar injuries, while 180 cat cases at four clinics during August and November 2011 included none. The 63 owners from the general public reported only one collar injury and no deaths in a lifetime of ownership, although 27% experienced collar incidents. In contrast, 22% reported cats needing treatment following road accidents, 53% reported cats needing treatment for fighting injuries and 62% had owned cats killed on the road. Most (62%) of the 55 respondents from a cat welfare society had experienced a collar incident, but only two cats needed treatment. One died. In contrast, 31 and 58% reported cats needing treatment for road accidents and fighting, respectively, and 41% had owned cats killed on the road. Fighting and road accidents are greater hazards to roaming cats than collars, which offer the compensatory benefits of mounting predation deterrents and ID tags.
Marine ecosystems are exposed to a wide variety of factors that may produce disturbances in their structure and functioning. The Gulf of California supports fisheries, tourism, intensive agriculture, mining, and more recently, shrimp aquaculture. Under such conditions, animals are forced to cope with several changes in their environment that can contribute to animal welfare problems. Serum cortisol level analysis may be a particularly useful means of assessing the physiological status of mammals potentially affected by increasing human activity in the Gulf of California, such as the California sea lion (Zalophus californianus californianus). In this study, we report for the first time the serum cortisol concentration of free-living, wild California sea lion pups. The analysis was performed in eleven rookeries along the Gulf of California. Two consecutive blood samples (S1 and S2) were obtained from 56 sea lion pups following a capture, handling and anaesthesia regime, and cortisol was measured by radioimmunoassay. Female pups showed higher serum cortisol than males in the first sample. In males, the second sample was significantly higher than the first. Cortisol levels in the two samples of both sexes combined differed between the Southern and Midriff-region rookeries. This information could be useful to assess welfare in wild populations of sea lions and to determine anthropogenic factors in the Gulf of California that may contribute to stress and reduced welfare.
Welfare is the state of an animal on a continuum, from poor to good, so many decisions about it are decisions of degree, such as how much feed, space or environmental enrichment should be provided. Other decisions are more discrete, such as whether animals should be kept in cages. However, in practice, many such decisions also involve a range of possibilities — such as whether laying hens should be kept in conventional cages, furnished cages, other housed systems or free range — so that decisions within the range are also of degree. Furthermore, in broader contexts, such as husbandry standards for farm animals, decisions are needed as to how many criteria are to be addressed, which are also decisions of degree. Similarly, decisions about which species to protect and from how early in individual development they need protection are to some extent categorical. This is sometimes referred to as ‘line drawing.’ However, this mainly refers to whether or not animals are sentient, and sentience is not clearly distinguished from other aspects of animals’ cognition and responses, so there is no conclusive boundary between ‘haves’ and ‘have nots.’ So, these decisions are also of degree: is there sufficient evidence to ‘move the line’ further? When there are pressures against change, such as financial cost, should welfare advocates ask for small or large changes? The answer to this question will depend upon circumstances. But discussion of different circumstances suggests that compromise, realism, gradualism and pragmatism are all important in achieving improvements in animal welfare, while noting that other tactics also contribute in particular contexts.
An online survey, using open and prompted response questions, was undertaken to collate the views of stakeholders on the priority welfare issues currently facing companion dogs (Canis familiaris) in Great Britain and on dogs’ general quality of life. The stakeholder sectors targeted broadly comprised Education, Government, Industry, Charity and Veterinary. Overall, respondents described companion dogs as, at minimum, having a life worth living. Whether welfare issues were openly described or ranked within a set list, those of high priority in the perceptions of stakeholders matched those cited in published scientific literature; particularly, exaggerated physical features, inherited disease, obesity and inappropriate socialisation. Puppy farming and status dogs, which have been highlighted recently in the media, were also viewed as important. Lack of appropriate mental stimulation, irresponsible ownership and inappropriate environment were raised as priority issues by stakeholders and are under-reported in scientific literature. Significant differences between stakeholder sectors in ranking of welfare issues perceived importance, urgency to rectify, impact (on the individual) or prevalence in Britain may be explained by vested interests, organisational roles, differences in terminology and the contexts within which stakeholders came into contact with companion dogs. Pet travel, dew claw removal and complementary and alternative medicines were amongst those issues thought to be of least urgent welfare concern. Issues perceived to enhance welfare included the quality of veterinary care, physical stimulation, educational resources, responsible ownership, the high status of dogs in society and the work of welfare organisations.
Gestation stall housing for pregnant sows (Sus scrofa) has been, or is being, phased out in many parts of the world in response to public criticism. However, in Brazil, one of the largest global producers and exporters of pork, gestation stall housing is still common. The objective of this study was to explore the views of Brazilians, including participants associated (ALP) or not with livestock production (NotALP), on gestation stall housing. Participants were provided the option of accessing a short text describing the housing system and a video of pregnant sows housed in either individual or group housing. Participants (ALP; n = 176, NotALP; n = 173) were asked to state their position on housing pregnant sows in individual stalls and to provide the reason(s) justifying their position. More NotALP (87%) participants than ALP (69%) participants rejected individual stalls. More participants (85%) that accessed the optional information rejected the stalls than those (71%) that did not. Qualitative analyses revealed that animal welfare, most often in reference to animal sentience, freedom of movement and ethics, was the main justification given for rejecting gestation stalls. Those in favour of individual stalls justified their position with statements such as improved production, handling and animal health, and reduced aggression. This qualitative, exploratory study, based on a convenience sample of participants, does not represent the views of Brazilian society; however, it identified some shared values between participants associated with livestock production and those that are not. Our findings highlight that opposition to gestation stalls for sows reflects an ethical position regarding the treatment of livestock and should not be interpreted as support for group housing in confined systems.
Surgical castration is a painful procedure that is routinely performed without pain relief on commercial pig (Sus scrofa domesticus) farms. Previous research has focused on quantifying piglet pain response through behaviours. However, to date, behavioural sampling methodologies used to quantify pain associated with castration have not been validated. Therefore, the objective of this study was to validate scan sampling methodologies (2-min, 3-min, 5-min, 10-min and 15-min intervals) to quantify piglet pain responses expressed by castrated piglets’ behaviour. A total of 39 Yorkshire-Landrace × Duroc male piglets (five days of age) were surgically castrated using a scalpel blade. Behaviour frequency and duration (scratching, spasms, stiffness, tail wagging and trembling) of each piglet were continuously collected for the first 15 min of the following hours relative to castration (-24, 1-8 and 24). To determine if the sampling interval accurately reflected true duration and frequency for each behaviour, as determined by continuous observation, criteria previously utilised from other behavioural validation studies were used: coefficient of determination above 0.9, slope not statistically different from one and intercept not statistically different from zero. No scan sampling interval provided accurate estimates for any behavioural indicators of pain. The results of this study suggest that continuous sampling is the most appropriate methodology to fully capture behaviour specific to pain associated with castration. Using validated behavioural methodologies in future research can assist in the development of objective, science-based protocols for managing pig pain.