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Many adopted children face challenges at school and college which adversely affect their experience and attainment. Without effective post-adoption support, they are at an increased risk of being excluded from or opting out of education. While this has been confirmed by research, there has been less discussion of the problems children face and the services they find helpful. This is especially the case for those over the age of 16 (the statutory school leaving age in the UK). To explore this, semi-structured interviews were conducted with five adopted young people aged between 16 and 19. A thematic analysis of their responses identified four factors that help them continue in post-16 education: (1) a positive self-identity; (2) supportive people; (3) supportive approaches; and (4) supportive systems. Participants identified the benefits of staff appreciating the needs of adopted children and the features that promote an ‘adoption-friendly’ ethos in schools and colleges. The implications of these observations for understanding the educational needs of adopted young people, practice development and future research are discussed. Attention is drawn to the importance of multi-agency work and the role of adopted young people in steering and co-producing effective support in educational settings.

This article presents a critical review of research into post-adoption support in educational settings using a rigorous systematic methodology. This is an important topic as effective support servi...

Summary: Background: Historical, colonial, and racist policies continue to influence the health of Indigenous people, and they continue to have higher rates of chronic diseases and reduced life expectancy compared with non-Indigenous people. We determined factors accounting for variations in cardiovascular risk factors among First Nations communities in Canada. Methods: Men and women (n=1302) aged 18 years or older from eight First Nations communities participated in a population-based study. Questionnaires, physical measures, blood samples, MRI of preclinical vascular disease, and community audits were collected. In this cross-sectional analysis, the main outcome was the INTERHEART risk score, a measure of cardiovascular risk factor burden. A multivariable model was developed to explain the variations in INTERHEART risk score among communities. The secondary outcome was MRI-detected carotid wall volume, a measure of subclinical atherosclerosis. Findings: The mean INTERHEART risk score of all communities was 17·2 (SE 0·2), and more than 85% of individuals had a risk score in the moderate to high risk range. Subclinical atherosclerosis increased significantly across risk score categories (p

AimsCOVID-19 has had a significant impact on healthcare provision, accessibility and psychiatric presentations. We aim to investigate the impact of the pandemic on psychiatric services and the severity of presentations in Edinburgh, with a particular focus on the North-West Edinburgh Community Mental Health Team (NW CMHT).MethodMeasures of the impact of the pandemic on NW CMHT were identified as referral numbers from primary care and Did Not Attend (DNA) rates. Royal Edinburgh Hospital admissions, detentions under the Mental Health (Care and Treatment) (Scotland) Act 2003 (MHA) and Out of Hours (OOH) contacts were used as proxy measures to explore the severity and urgency of presentations.Quantitative data focussing on these parameters for patients aged 18–65 years in NW CMHT in 2019 and 2020 were collected from NHS Lothian Analytical Services. OOH data were only available Edinburgh-wide. All data were anonymised in line with NHS Lothian Information Governance Policy.In order to assess the impact on staff, a questionnaire was created and disseminated, with qualitative data returned anonymously.ResultReferrals to NW CMHT decreased by 9.3% in 2020 (n = 2164) compared to 2019 (n = 2366). Referrals in April (n = 81) and May (n = 102) 2020 were far below the monthly average across the two years (n = 188).Appointment numbers were very similar in 2019 (n = 3542) and 2020 (n = 3514). Despite this, DNA and cancellation rates decreased by 3.94% in 2020. Questionnaire results illustrated some of the challenges for staff of working during a pandemic.Admissions to hospital reduced by 6.8% in 2020 (n = 219 vs n = 235). While MHA detentions in NW Edinburgh increased by only 1.8% (n = 173 vs n = 170), new Compulsory Treatment Orders (CTO) increased by 60%. Furthermore, OOH contacts across Edinburgh increased by 45.2% when compared to 2019.ConclusionThe COVID-19 pandemic altered the way patients accessed healthcare. Uncertainty of the public in accessing primary care services early in the pandemic may have contributed to reduced referral numbers.The increase in CTOs is suggestive of severe relapses in previously stable patients or new episodes of illness. The pandemic may have contributed to a reduction in early recognition, and referral, of those with major mental disorders resulting in more protracted or severe illness episodes. The increase in OOH crisis contacts supports such a hypothesis.Despite what would be expected, DNA and cancellation rates in NW CMHT reduced. The contribution of telemedicine to this warrants further exploration as a means of delivering healthcare in an efficient and accessible way.

Background & aims Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease characterized by destruction of intrahepatic bile ducts, portal inflammation and cirrhosis. Although rare in most populations, it is prevalent and often familial in British Columbia First Nations. We hypothesized that major genetic factors increased the risk in First Nations. Methods In all, 44 individuals with Primary Biliary Cholangitis and 61 unaffected relatives from 32 First Nations families participated. Family history and co-morbidities were documented. Medical records were reviewed and available biopsies were re-reviewed by our team pathologist. Genotyping was performed on DNA from 36 affected persons and 27 unaffected relatives using the Affymetrix Human Mapping 500K Array Set. MERLIN software was used to carry out multipoint parametric and nonparametric linkage analysis. Candidate genes were identified and entered into InnateDB and KEGG software to identify potential pathways affecting pathogenesis. Results In all, 34% of families were multiplex. Fifty per cent of cases and 33% of unaffected relatives reported other autoimmune disease. Three genomic regions (9q21, 17p13 and 19p13) produced LOD scores of 2.3 or greater suggestive of linkage, but no single linkage peak reached statistical significance. Candidate genes identified in the three regions suggested involvement of IL17, NFκB, IL6, JAK-STAT, IFNγ and TGFβ immune signalling pathways. Specifically, four genes-ACT1, PIN1, DNMT1 and NTN1-emerged as having roles in these pathways that may influence Primary Biliary Cholangitis pathogenesis. Conclusions Our whole genome linkage study results reflect the multifactorial nature of Primary Biliary Cholangitis, support previous studies suggesting signalling pathway involvement and identify new candidate genes for consideration.

Background: This is the first national indigenous cohort study in which a common, in-depth protocol with a common set of objectives has been adopted by several indigenous communities across Canada. Objectives: The overarching objective of the Canadian Alliance for Healthy Hearts and Minds (CAHHM) cohort is to investigate how the community-level environment is associated with individual health behaviors and the presence and progression of chronic disease risk factors and chronic diseases such as cardiovascular disease (CVD) and cancer. Methods: CAHHM aims to recruit approximately 2,000 First Nations indigenous individuals from up to nine communities across Canada and have participants complete questionnaires, blood collection, physical measurements, cognitive assessments, and magnetic resonance imaging (MRI). Results: Through individual- and community-level data collection, we will develop an understanding of the specific role of the socioenvironmental, biological, and contextual factors have on the development of chronic disease risk factors and chronic diseases. Conclusions: Information collected in the indigenous cohort will be used to assist communities to develop local management strategies for chronic disease, and can be used collectively to understand the contextual, environmental, socioeconomic, and biological determinants of differences in health status in harmony with First Nations beliefs and reality.

BackgroundVariable expressivity and incomplete penetrance between individuals with identical long QT syndrome (LQTS) causative mutations largely remain unexplained. Founder populations provide a unique opportunity to explore modifying genetic effects. We examined the role of a novel synonymousKCNQ1p.L353L variant on the splicing of exon 8 and on heart rate corrected QT interval (QTc) in a population known to have a pathogenic LQTS type 1 (LQTS1) causative mutation, p.V205M, inKCNQ1-encoded Kv7.1.Methods419 adults were genotyped for p.V205M, p.L353L and a previously described QTc modifier (KCNH2-p.K897T). Adjusted linear regression determined the effect of each variant on QTc, alone and in combination. In addition, peripheral blood RNA was extracted from three controls and three p.L353L-positive individuals. The mutant transcript levels were assessed via qPCR and normalised to overallKCNQ1transcript levels to assess the effect on splicing.ResultsFor women and men, respectively, p.L353L alone conferred a 10.0 (p=0.064) ms and 14.0 (p=0.014) ms increase in QTc and in men only a significant interaction effect in combination with the p.V205M (34.6 ms, p=0.003) resulting in a QTc of ∼500 ms. The mechanism of p.L353L's effect was attributed to approximately threefold increase in exon 8 exclusion resulting in ∼25% mutant transcripts of the totalKCNQ1transcript levels.ConclusionsOur results provide the first evidence that synonymous variants outside the canonical splice sites inKCNQ1can alter splicing and clinically impact phenotype. Through this mechanism, we identified that p.L353L can precipitate QT prolongation by itself and produce a clinically relevant interactive effect in conjunction with other LQTS variants.

Background— Long QT syndrome confers susceptibility to ventricular arrhythmia, predisposing to syncope, seizures, and sudden death. While rare globally, long QT syndrome is ≈15× more common in First Nations of Northern British Columbia largely because of a known mutation in KCNQ1 . However, 2 large multigenerational families were affected, but negative for the known mutation. Methods and Results— Long QT syndrome panel testing was carried out in the index case of each family, and clinical information was collected. Cascade genotyping was performed. Biochemical and myocyte-based assays were performed to evaluate the identified gene variant for loss-of-function activity. Index cases in these 2 families harbored a novel ANK2 c.1937C>T variant (p.S646F). An additional 16 carriers were identified, including 2 with structural heart disease: one with cardiomyopathy resulting in sudden death and the other with congenital heart disease. For all carriers of this variant, the average QTc was 475 ms (±40). Although ankyrin-B p.S646F is appropriately folded and expressed in bacteria, the mutant polypeptide displays reduced expression in cultured H9c2 cells and aberrant localization in primary cardiomyocytes. Furthermore, myocytes expressing ankyrin-B p.S646F lack normal membrane targeting of the ankyrin-binding partner, the Na/Ca exchanger. Thus, ankyrin-B p.S646F is a loss-of-function variant. Conclusions— We identify the first disease-causing ANK2 variant localized to the membrane-binding domain resulting in reduced ankyrin-B expression and abnormal localization. Further study is warranted on the potential association of this variant with structural heart disease given the role of ANK2 in targeting and stabilization of key structural and signaling molecules in cardiac cells.

Carnitine palmitoyltransferase 1A (CPT1A), encoded by the gene CPT1A, is the hepatic isoform of CPT1 and is a major regulatory point in long-chain fatty acid oxidation. CPT1A deficiency confers risk for hypoketotic hypoglycaemia, hepatic encephalopathy, seizures, and sudden unexpected death in infancy (SUDI). It remains controversial whether the CPT1A gene variant, c.1436C>T (p.P479L), identified in Inuit, First Nations, and Alaska Native infants, causes susceptibility to decompensation, in particular during times of fever and intercurrent illness. Although newborn screening for the P479L variant occurs in some jurisdictions, background knowledge about the presence of the variant in Canadian Aboriginal populations is lacking. In an effort to understand the population implications of the variant in northern Canada, overall frequencies of the variant were assessed. Further studies are underway to determine associated risk. Ethics approval was obtained from university REBs, local research institutes, and with consultation with territorial Aboriginal groups. Newborn screening blood spots from all infants born in 2006 in the three territories were genotyped for the p.P479L variant. p.P479L (c.1436C>T) allele frequencies in the three territories were 0.02, 0.08, and 0.77 in Yukon ( n =325), Northwest Territories ( n =564), and Nunavut ( n =695), respectively. Homozygosity rates were 0%, 3%, and 64%. Aboriginal status was available only in NWT, with allele frequencies of 0.04, 0.44, 0.00, and 0.01 for First Nations, Inuvialuit/Inuit, Metis, and non-Aboriginal populations. Although individual blood spots were not identified for Aboriginal ethnicity in Nunavut infants, ~90% of infants in Nunavut are born to Inuit women. The allele frequency and rate of homozygosity for the CPT1A P479L variant were high in Inuit and Inuvialuit who reside in northern coastal regions. The variant is present at a low frequency in First Nations populations, who reside in areas less coastal than the Inuit or Inuvialuit in the two western territories. The significance of the population and geographic distribution remains unclear, but the high population frequencies of the variant suggest a historically low penetrance for adverse outcomes. Further evidence is needed to determine if there is an increased risk for infant mortality and morbidity and whether newborn screening will be indicated on a population basis.

Objectives. Little is known about the rates of congenital anomalies in the northernmost regions of the world. As in other parts of the world, it is crucial to assess the relative rates and trends of adverse birth outcomes and birth defects, as indicators of population health and to develop public health strategies for prevention. The aim of this review is to catalogue existing and developing birth outcome and birth defect surveillance within and around the geographic jurisdiction of the International Union of Circumpolar Health (IUCH). Study design. Descriptive study. Methods. The representatives of the IUCH Birth Defects Working Group catalogued existing and developing birth and birth defect surveillance systems and the extent of information they contain to determine inter-regional comparability. Results. Systematic population-based registration of birth outcomes including birth defects occurs to some degree in all circumpolar countries, but the quality of collection and the coverage in northernmost regions vary. There are limited circumpolar jurisdictions with surveillance systems collecting birth defect information beyond the perinatal period. Efforts are underway in Canada and Russia to improve the quality and comprehensiveness of the information collected in the northern regions. Conclusions. Although there is variability in the comprehensiveness of information collected in northern jurisdictions limiting sophisticated comparative analyses between regions, there is untapped potential for baseline analyses of specific risks and outcomes that could provide insight into geographic differences and gaps in surveillance that could be improved. (Int J Circumpolar Health 2009; 68(5): 443-458) Keywords: Arctic, circumpolar, birth defects, medical birth registries, congenital anomalies surveillance

The use of judicial performance evaluation is becoming more prevalent in the U.S. and in foreign jurisdictions. While such evaluations are indeed growing in prevalence, the federal judiciary has been largely resistant to such a program. The current study, through a survey of attorneys who have appeared before an Article III federal judge within the last three years, seeks to examine attitudes towards the implementation of a formal federal judicial performance evaluation program. Overall results indicate mixed attitudes towards implementing a federal performance evaluation program. Specific results for those for or against an evaluation program are discussed in detail.

Objectives. Nunavut is the most northerly jurisdiction in Canada of which 85% of inhabitants are Inuit. Although most infants are born healthy, Nunavut leads the country for adverse early child health outcomes such as infant mortality, rates of birth defects, prematurity and low birth weight. Public health and community efforts are needed to understand and improve outcomes. Methods. To inform these issues, a combined University of British Columbia/Nunavut Public Health Strategy effort has initiated a comprehensive maternal–child health surveillance system (from 16 weeks gestation to age 5). A diverse group of professional and lay stakeholders were brought together initially to determine local interest. Following this, a series of small working groups were held to decide on potential prenatal, perinatal and early child health variables, to be documented. Results. Over 100 Nunavut participants have now had some role in the development of the system which has been initiated. Pre-existing standard prenatal forms and well-child assessment forms have been modified to include “Nunavut specific” variables of nutrition, food and domestic security, exposures in pregnancy, birth defects, development, chronic diseases of childhood and paternal information. Conclusion. This comprehensive maternal–child health information system has been developed with the extensive input of health care providers and stakeholders, utilizing community and public health systems already in place. Careful assessment of local needs has contributed to database development, privacy protection, potential data utilization for health promotion and plans for dissemination of findings. It is hoped that this will be a user-friendly surveillance system, adaptable to other community and public health systems that will improve the understanding of Aboriginal maternal–child health determinants. (Int J Circumpolar Health 2011; 70(4):363-372) Keywords: Nunavut, maternal–child health, birth defects, infant mortality, surveillance

Exposure of a sunflower leaf to hypoxia or chilling stress interrupts translocation from the leaf and also alters the distribution of 14C-assimilates into sugars and amino acids. As translocation from the leaf recovers, there are changes in the distribution of label in the sugars and amino acids that coincide with this recovery. These observations lend support to the idea that certain concentrations of sugars and amino acids are necessary to support translocation out of the leaf.

This paper examines experiences in developing, supporting and sustaining community-based enterprises that are based on the use of forest, coastal and marine resources in the insular Caribbean, with a focus on the eastern Caribbean, and draws lessons from that experience. The three cases reviewed include community-based enterprises involved in forest conservation, turtle protection, tour guiding and fisheries. Other experiences are also used to inform the lessons and conclusions of the paper. The paper first identifies some of the cultural, social or economic factors that have favoured or hindered the development of these enterprises, highlighting the conditions that are specific to Caribbean societies, where the majority of the people have throughout history been denied access to valuable natural resources, where production is largely driven by external markets, and where there is not a long tradition of community natural resource management. It then examines the extent to which the economic success of individual business ventures contributes to or hinders the achievement of the broader community social, political, cultural and environmental goals. On the basis of these analyses, the paper proposes a number of enabling conditions that may be required, in the context of the insular Caribbean, for these initiatives to flourish.