Loss-of-function variants in JPH1 cause congenital myopathy with prominent facial involvement.

Background: Weakness of facial, ocular, and axial muscles is a common clinical presentation in congenital myopathies caused by pathogenic variants in genes encoding triad proteins. Abnormalities in triad structure and function resulting in disturbed excitation-contraction coupling and Ca 2+ homeostasis can contribute to disease pathology. Methods: We analysed exome and genome sequencing data from three unrelated individuals with congenital myopathy characterised by striking facial, ocular, and bulbar involvement. We collected deep phenotypic data from the affected individuals. We analysed the RNA-seq data of one proband and performed gene expression outlier analysis in 129 samples. Results: The three probands had remarkably similar clinical presentation with prominent facial, ocular, and bulbar features. Disease onset was in the neonatal period with hypotonia, poor feeding, cleft palate and talipes. Muscle weakness was generalised but most prominent in the lower limbs with facial weakness also present. All patients had myopathic facies, bilateral ptosis, ophthalmoplegia and fatiguability. While muscle biopsy on light microscopy did not show any obvious morphological abnormalities, ultrastructural analysis showed slightly reduced triads, and structurally abnormal sarcoplasmic reticulum. DNA sequencing identified three unique homozygous loss of function variants in JPH1 , encoding junctophilin-1 in the three families; a stop-gain (c.354C>A; p.Tyr118*) and two frameshift (c.373del p.Asp125Thrfs*30 and c.1738del; p.Leu580Trpfs*16) variants. Muscle RNA-seq showed strong downregulation of JPH1 in the F3 proband. Conclusions: Junctophilin-1 is critical to the formation of skeletal muscle triad junctions by connecting the sarcoplasmic reticulum and T-tubules. Our findings suggest that loss of JPH1 results in a congenital myopathy with prominent facial, bulbar and ocular involvement. Key message: This study identified novel homozygous loss-of-function variants in the JPH1 gene, linking them to a unique form of congenital myopathy characterised by severe facial and ocular symptoms. Our research sheds light on the critical impact on junctophilin-1 function in skeletal muscle triad junction formation and the consequences of its disruption resulting in a myopathic phenotype. What is already known on this topic: Previous studies have shown that pathogenic variants in genes encoding triad proteins lead to various myopathic phenotypes, with clinical presentations often involving muscle weakness and myopathic facies. The triad structure is essential for excitation-contraction (EC) coupling and calcium homeostasis and is a key element in muscle physiology. What this study adds and how this study might affect research practice or policy: This study establishes that homozygous loss-of-function mutations in JPH1 cause a congenital myopathy predominantly affecting facial and ocular muscles. This study also provides clinical insights that may aid the clinicians in diagnosing similar genetically unresolved cases.

Data from a national survey of United States primary care physicians on genetic risk scores for common disease prevention.

Genetic risk scores (GRS) are an emerging and rapidly evolving genomic medicine innovation that may contribute to more precise risk stratification for disease prevention. Inclusion of GRS in routine medical care is imminent, and understanding how physicians perceive and intend to utilize GRS in practice is an important first step in facilitating uptake. This dataset was derived from an electronic survey and comprises one of the first, largest, and broadest samples of United States primary care physician perceptions on the clinical decision-making, benefits, barriers, and utility of GRS to date. The dataset is nearly complete (<1% missing data) and contains responses from 369 PCPs spanning 58 column variables. The public repository includes minimally filtered, de-identified data, all underlying survey versions and items, a data dictionary, and associated analytic files.

The GenoVA study: Equitable implementation of a pragmatic randomized trial of polygenic-risk scoring in primary care.

Polygenic risk scores (PRSs) hold promise for disease risk assessment and prevention. The Genomic Medicine at Veterans Affairs (GenoVA) Study is addressing three main challenges to the clinical implementation of PRSs in preventive care: defining and determining their clinical utility, implementing them in time-constrained primary care settings, and countering their potential to exacerbate healthcare disparities. The study processes used to test patients, report their PRS results to them and their primary care providers (PCPs), and promote the use of those results in clinical decision-making are modeled on common practices in primary care. The following diseases were chosen for their prevalence and familiarity to PCPs: coronary artery disease; type 2 diabetes; atrial fibrillation; and breast, colorectal, and prostate cancers. A randomized clinical trial (RCT) design and primary outcome of time-to-new-diagnosis of a target disease bring methodological rigor to the question of the clinical utility of PRS implementation. The study's pragmatic RCT design enhances its relevance to how PRS might reasonably be implemented in primary care. Steps the study has taken to promote health equity include the thoughtful handling of genetic ancestry in PRS construction and reporting and enhanced recruitment strategies to address underrepresentation in research participation. To date, enhanced recruitment efforts have been both necessary and successful: participants of underrepresented race and ethnicity groups have been less likely to enroll in the study than expected but ultimately achieved proportional representation through targeted efforts. The GenoVA Study experience to date offers insights for evaluating the clinical utility of equitable PRS implementation in adult primary care.

Dissemination and implementation of clinical practice guidelines: a longitudinal, mixed-methods evaluation of the Canadian Task Force on Preventive Health Care's knowledge translation efforts.

BACKGROUND: The Canadian Task Force on Preventive Health Care (task force) develops evidence-based preventive health care guidelines and knowledge translation (KT) tools to facilitate guideline dissemination and implementation. We aimed to determine practitioners' awareness of task force guidelines and KT tools and explore barriers and facilitators to their use. METHODS: The task force's KT team completed annual evaluations using surveys and interviews with primary care providers in Canada from 2014 to 2020, to assess practitioners' awareness and determinants of use of task force guidelines and tools. We transcribed interviews verbatim and double-coded them using a framework analysis approach. RESULTS: A total of 1284 primary care practitioners completed surveys and 183 participated in interviews. On average, 79.9% of participants were aware of the task force's 7 cancer screening guidelines, 36.2% were aware of the other 6 screening guidelines and 18.6% were aware of the 3 lifestyle or prevention guidelines. Participants identified 13 barriers and 7 facilitators to guideline and KT tool implementation; these were consistent over time. Participants identified strategies at the public and patient, provider and health systems levels to improve uptake of guidelines. INTERPRETATION: Canadian primary care practitioners were more aware of task force cancer screening guidelines than its other preventive health guidelines. Over the 6-year period, participants consistently reported barriers to guideline uptake, including misalignment with patient preferences and other provincial or specialty guideline organizations. Further evaluations will assess tailored strategies to address the barriers identified.

Activating hidden signals by mimicking cryptic sites in a synthetic extracellular matrix.

Cryptic sites are short signaling peptides buried within the native extracellular matrix (ECM). Enzymatic cleavage of an ECM protein reveals these hidden peptide sequences, which interact with surface receptors to control cell behavior. Materials that mimic this dynamic interplay between cells and their surroundings via cryptic sites could enable application of this endogenous signaling phenomenon in synthetic ECM hydrogels. We demonstrate that depsipeptides ("switch peptides") can undergo enzyme-triggered changes in their primary sequence, with proof-of-principle studies showing how trypsin-triggered primary sequence rearrangement forms the bioadhesive pentapeptide YIGSR. We then engineered cryptic site-mimetic synthetic ECM hydrogels that experienced a cell-initiated gain of bioactivity. Responding to the endothelial cell surface enzyme aminopeptidase N, the inert matrix transformed into an adhesive synthetic ECM capable of supporting endothelial cell growth. This modular system enables dynamic reciprocity in synthetic ECMs, reproducing the natural symbiosis between cells and their matrix through inclusion of tunable hidden signals.

Effect of Perioperative Palliative Care on Health-Related Quality of Life Among Patients Undergoing Surgery for Cancer: A Randomized Clinical Trial.

Importance: Involvement of palliative care specialists in the care of medical oncology patients has been repeatedly observed to improve patient-reported outcomes, but there is no analogous research in surgical oncology populations. Objective: To determine whether surgeon-palliative care team comanagement, compared with surgeon team alone management, improves patient-reported perioperative outcomes among patients pursuing curative-intent surgery for high morbidity and mortality upper gastrointestinal (GI) cancers. Design, Setting, and Participants: From October 20, 2018, to March 31, 2022, a patient-randomized clinical trial was conducted with patients and clinicians nonblinded but the analysis team blinded to allocation. The trial was conducted in 5 geographically diverse academic medical centers in the US. Individuals pursuing curative-intent surgery for an upper GI cancer who had received no previous specialist palliative care were eligible. Surgeons were encouraged to offer participation to all eligible patients. Intervention: Surgeon-palliative care comanagement patients met with palliative care either in person or via telephone before surgery, 1 week after surgery, and 1, 2, and 3 months after surgery. For patients in the surgeon-alone group, surgeons were encouraged to follow National Comprehensive Cancer Network-recommended triggers for palliative care consultation. Main Outcomes and Measures: The primary outcome of the trial was patient-reported health-related quality of life at 3 months following the operation. Secondary outcomes were patient-reported mental and physical distress. Intention-to-treat analysis was performed. Results: In total, 359 patients (175 [48.7%] men; mean [SD] age, 64.6 [10.7] years) were randomized to surgeon-alone (n = 177) or surgeon-palliative care comanagement (n = 182), with most patients (206 [57.4%]) undergoing pancreatic cancer surgery. No adverse events were associated with the intervention, and 11% of patients in the surgeon-alone and 90% in the surgeon-palliative care comanagement groups received palliative care consultation. There was no significant difference between study arms in outcomes at 3 months following the operation in patient-reported health-related quality of life (mean [SD], 138.54 [28.28] vs 136.90 [28.96]; P = .62), mental health (mean [SD], -0.07 [0.87] vs -0.07 [0.84]; P = .98), or overall number of deaths (6 [3.7%] vs 7 [4.1%]; P > .99). Conclusions and Relevance: To date, this is the first multisite randomized clinical trial to evaluate perioperative palliative care and the earliest integration of palliative care into cancer care. Unlike in medical oncology practice, the data from this trial do not suggest palliative care-associated improvements in patient-reported outcomes among patients pursuing curative-intent surgeries for upper GI cancers. Trial Registration: ClinicalTrials.gov Identifier: NCT03611309.

Primary care physician use of patient race and polygenic risk scores in medical decision-making.

PURPOSE: The use of patient race in medicine is controversial for its potential either to exacerbate or address health disparities. Polygenic risk scores (PRSs) have emerged as a tool for risk stratification models used in preventive medicine. We examined whether PRS results affect primary care physician (PCP) medical decision-making and whether that effect varies by patient race. METHODS: Using an online survey with a randomized experimental design among PCPs in a national database, we ascertained decision-making around atherosclerotic cardiovascular disease prevention and prostate cancer screening for case scenario patients who were clinically identical except for randomized reported race. RESULTS: Across 369 PCPs (email open rate = 10.8%, partial completion rate = 93.7%), recommendations varied with PRS results in expected directions (low-risk results, no available PRS results, and high-risk results). Still, physicians randomized to scenarios with Black patients were more likely to recommend statin therapy than those randomized to scenarios with White patients (odds ratio = 1.74, 95% CI = 1.16-2.59, P = .007) despite otherwise identical clinical profiles and independent of PRS results. Similarly, physicians were more likely to recommend prostate cancer screening for Black patients than for White patients (odds ratio = 1.58, 95% CI = 1.06-2.35, P = .025) despite otherwise identical clinical and genetic profiles. CONCLUSION: Despite advances in precision risk stratification, physicians will likely continue to use patient race implicitly or explicitly in medical decision-making.

Interventions to address potentially inappropriate prescriptions and over-the-counter medication use among adults 65 years and older in primary care settings: protocol for a systematic review.

PURPOSE: To inform recommendations by the Canadian Task Force on Preventive Health Care on potentially inappropriate prescribing and over-the-counter (OTC) medication use among adults aged 65 years and older in primary care settings. This protocol outlines the planned scope and methods for a systematic review of the benefits and harms and acceptability of interventions to reduce potentially inappropriate prescriptions and OTC medication use. METHODS: De novo systematic reviews will be conducted to synthesize the available evidence on (a) the benefits and harms of interventions to reduce potentially inappropriate prescriptions and OTC medications compared to no intervention, usual care, or non- or minimally active intervention among adults aged 65 years and older and (b) the acceptability of these interventions or attributes among patients. Outcomes of interest for the benefits and harms review are all-cause mortality, hospitalization, non-serious adverse drug reactions, quality of life, emergency department visits, injurious falls, medical visits, and the number of medications (and number of pills). Outcomes for the acceptability review are the preference for and relative importance of different interventions or their attributes. For the benefits and harms review, we will search MEDLINE, Embase, and Cochrane Central Register of Controlled Trials for randomized controlled trials. For the acceptability review, we will search MEDLINE, Embase, PsycInfo, Cochrane Central Register of Controlled Trials, and the NHS Economic Evaluation Database for experimental and observational studies with a comparator. Websites of relevant organizations, other grey literature sources, and reference lists of included studies and reviews will be searched. Title and abstract screening will be completed by two independent reviewers using the liberal accelerated approach. Full-text review, data extraction, risk of bias assessments, and GRADE (Grading of Recommendations Assessment, Development and Evaluation) will be completed independently by two reviewers, with any disagreements resolved by consensus or by consulting with a third reviewer. The GRADE approach will be used to assess the certainty of the evidence for outcomes. DISCUSSION: The results of this systematic review will be used by the Canadian Task Force on Preventive Health Care to inform their recommendation on potentially inappropriate prescribing and OTC medication use among adults aged 65 years and older. SYSTEMATIC REVIEW REGISTRATION: PROSPERO (KQ1: CRD42022302313; KQ2: CRD42022302324); Open Science Framework ( https://osf.io/urj4b/ ).

Screening for prostate cancer: protocol for updating multiple systematic reviews to inform a Canadian Task Force on Preventive Health Care guideline update.

PURPOSE: To inform updated recommendations by the Canadian Task Force on Preventive Health Care on screening for prostate cancer in adults aged 18 years and older in primary care. This protocol outlines the planned scope and methods for a series of systematic reviews. METHODS: Updates of two systematic reviews and a de novo review will be conducted to synthesize the evidence on the benefits and harms of screening for prostate cancer with a prostate-specific antigen (PSA) and/or digital rectal examination (DRE) (with or without additional information) and patient values and preferences. Outcomes for the benefits of screening include reduced prostate cancer mortality, all-cause mortality, and incidence of metastatic prostate cancer. Outcomes for the harms of screening include false-positive screening tests, overdiagnosis, complications due to biopsy, and complications of treatment including incontinence (urinary or bowel), and erectile dysfunction. The quality of life or functioning (overall and disease-specific) and psychological effects outcomes are considered as a possible benefit or harm. Outcomes for the values and preferences review include quantitative or qualitative information regarding the choice to screen or intention to undergo screening. For the reviews on benefits or harms, we will search for randomized controlled trials, quasi-randomized, and controlled studies in MEDLINE, Embase, and the Cochrane Central Register of Controlled Trials. For the review on values and preferences, we will search for experimental or observational studies in MEDLINE, Embase, and PsycInfo. For all reviews, we will also search websites of relevant organizations, gray literature, and reference lists of included studies. Title and abstract screening, full-text review, data extraction, and risk of bias assessments will be completed independently by pairs of reviewers with any disagreements resolved by consensus or by consulting with a third reviewer. The GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach will be used to assess the certainty of the evidence for each outcome. DISCUSSION: The series of systematic reviews will be used by the Canadian Task Force on Preventive Health Care to update their 2014 guideline on screening for prostate cancer in adults aged 18 years and older. Systematic review registration This review has been registered with PROSPERO (CRD42022314407) and is available on the Open Science Framework (osf.io/dm32k).

Performance of EHR classifiers for patient eligibility in a clinical trial of precision screening.

BACKGROUND: Validated computable eligibility criteria use real-world data and facilitate the conduct of clinical trials. The Genomic Medicine at VA (GenoVA) Study is a pragmatic trial of polygenic risk score testing enrolling patients without known diagnoses of 6 common diseases: atrial fibrillation, coronary artery disease, type 2 diabetes, breast cancer, colorectal cancer, and prostate cancer. We describe the validation of computable disease classifiers as eligibility criteria and their performance in the first 16 months of trial enrollment. METHODS: We identified well-performing published computable classifiers for the 6 target diseases and validated these in the target population using blinded physician review. If needed, classifiers were refined and then underwent a subsequent round of blinded review until true positive and true negative rates ≥80% were achieved. The optimized classifiers were then implemented as pre-screening exclusion criteria; telephone screens enabled an assessment of their real-world negative predictive value (NPV-RW). RESULTS: Published classifiers for type 2 diabetes and breast and prostate cancer achieved desired performance in blinded chart review without modification; the classifier for atrial fibrillation required two rounds of refinement before achieving desired performance. Among the 1077 potential participants screened in the first 16 months of enrollment, NPV-RW of the classifiers ranged from 98.4% for coronary artery disease to 99.9% for colorectal cancer. Performance did not differ by gender or race/ethnicity. CONCLUSIONS: Computable disease classifiers can serve as efficient and accurate pre-screening classifiers for clinical trials, although performance will depend on the trial objectives and diseases under study.

STIM1 and ORAI1 mutations leading to tubular aggregate myopathies are sensitive to the Store-operated Ca2+-entry modulators CIC-37 and CIC-39.

Gain-of-function mutations on STIM1 and ORAI1 genes are responsible for an increased store-operated calcium entry, and underlie the characteristic symptoms of three overlapping ultra-rare genetic disorders (i.e tubular aggregate myopathy, Stormorken syndrome, York platelet syndrome) that can be grouped as tubular aggregate myopathies. These mutations lead to a wide spectrum of defects, which usually include muscle weakness and cramps. Negative modulators of store-operated Ca2+-entry targeting wild-type STIM1 and ORAI1 have entered clinical trials for a different array of disorders, including pancreatitis, COVID-19, cancer, and autoimmune disorders and, while efficacy data is awaited, safety data indicates tolerability of this STIM1/ORAI1 mutations are amenable to pharmacological intervention. If this were so, given that there are no approved treatments or clinical trials ongoing for these rare disorders, it could be envisaged that these agents could also rehabilitate tubular aggregate myopathy patients. In the present contribution we characterized the Ca2+-entry patterns induced by eleven STIM1 and three ORAI1 mutations in heterologous systems or in patient-derived cells, i.e. fibroblasts and myotubes, and evaluated the effect of CIC-37 and CIC-39, two novel store-operated calcium entry modulators. Our data show that all STIM1 and ORAI1 gain-of-function mutations tested, with the possible exception of the R304Q STIM1 mutation, are amenable to inhibition, albeit with slightly different sensitivities, paving the way to the development of SOCE modulators in tubular aggregate myopathies.

Identification of LAMA1 mutations ends diagnostic odyssey and has prognostic implications for patients with presumed Joubert syndrome.

Paediatric neurology syndromes are a broad and complex group of conditions with a large spectrum of clinical phenotypes. Joubert syndrome is a genetically heterogeneous neurological ciliopathy syndrome with molar tooth sign as the neuroimaging hallmark. We reviewed the clinical, radiological and genetic data for several families with a clinical diagnosis of Joubert syndrome but negative genetic analysis. We detected biallelic pathogenic variants in LAMA1, including novel alleles, in each of the four cases we report, thereby establishing a firm diagnosis of Poretti-Boltshauser syndrome. Analysis of brain MRI revealed cerebellar dysplasia and cerebellar cysts, associated with Poretti-Boltshauser syndrome and the absence of typical molar tooth signs. Using large UK patient cohorts, the relative prevalence of Joubert syndrome as a cause of intellectual disability was 0.2% and of Poretti-Boltshauser syndrome was 0.02%. We conclude that children with congenital brain disorders that mimic Joubert syndrome may have a delayed diagnosis due to poor recognition of key features on brain imaging and the lack of inclusion of LAMA1 on molecular genetic gene panels. We advocate the inclusion of LAMA1 genetic analysis on all intellectual disability and Joubert syndrome gene panels and promote a wider awareness of the clinical and radiological features of these syndromes.

Community Health Workers as Healthcare Navigators in Primary Care Chronic Disease Management: a Systematic Review.

BACKGROUND: This review was carried out to synthesize the evidence of the effectiveness of community health worker (CHW) navigation in primary care chronic disease management. METHODS: We searched the English language literature between January 1990 and March 2020 in Medline, Embase, Emcare, PubMed, Psych Info, CINAHL, Scopus, and Medline Epub ahead of print. Data extraction, quality rating, and assessment of the reporting of interventions were performed by two reviewers independently and the findings were synthesized narratively. RESULTS: Twenty-nine articles met the inclusion criteria. All but two were carried out in the USA and half were randomized controlled trials. Six of the 29 studies were of strong methodological quality while 12 were moderate and 11 weak. Overall, CHW navigation interventions were effective in increasing adherence to cancer screening and improving use of primary care for chronic disease management. There was insufficient evidence that they improved clinical outcomes or risk factors and reduced use of secondary or tertiary care or that they were cost-effective. However, criteria for recruitment, duration, and mode of training and supervision arrangements varied greatly between studies. DISCUSSION: CHW navigation interventions improved aspects of chronic disease management. However, there is insufficient evidence of the impact on patient experience, clinical outcomes, or cost-effectiveness of the interventions. Future research should focus on standardizing organizational components of the CHW navigation interventions and evaluating their cost-effectiveness. PROTOCOL REGISTRATION: The review protocol was published in PROSPERO (CRD42020153921).

Relative burden of lung and pleural cancers from exposure to asbestos: a cross-sectional analysis of occupational mortality in England and Wales.

OBJECTIVES: To explore the extent to which asbestos-exposed jobs vary in the ratio of excess mortality from lung cancer to deaths from pleural cancer. DESIGN: Using data on underlying cause of death and last full-time occupation for 3 688 916 deaths among men aged 20-74 years in England and Wales during 1979-2010, we calculated proportional mortality ratios (PMRs), standardised for age and social class, with all occupations combined as reference. For each of 22 asbestos-exposed job groups with significantly elevated PMRs for pleural cancer, we calculated excess mortality from lung cancer (observed minus expected deaths) and its ratio to number of deaths from pleural cancer. To reduce confounding effects of smoking, we adjusted expected deaths from lung cancer in each job group, according to a formula based on its PMR for chronic obstructive pulmonary disease. SETTING: England and Wales. PARTICIPANTS: 3 688 916 men who died aged 20-74 years during 1979-2010. OUTCOME MEASURES: Ratios of excess mortality from lung cancer to deaths from pleural cancer by job group. RESULTS: Adjusted PMRs for lung cancer were elevated in all but 4 of the 22 asbestos-exposed job groups, but the ratio of excess lung cancer to deaths from pleural cancer varied widely between job groups, being significantly greater than the overall ratio in six, and significantly less in seven. Analysis for 2001-2010, when (because of changes in coding) ascertainment of pleural tumours was more reliable, showed similar variation between job groups, and indicated an overall ratio of 0.28. CONCLUSIONS: Excess lung cancer in asbestos-exposed jobs is not in a simple proportion to deaths from pleural cancer, and the ratio may vary importantly according to intensity of exposure to different types of asbestos and concomitant smoking habits. The current burden of lung cancer from occupational exposure to asbestos in Britain may not be so high as previously thought.

Motivos da adesão de idosos às Academias da Terceira Idade / Reasons for the adherence of older adults to Gyms for Seniors

Resumo Verificar os motivos de adesão (ingresso e permanência) de idosos às Academias da Terceira Idade (ATI) da cidade do Rio de Janeiro, RJ, Brasil. Método: Foram entrevistados 396 idosos de ambos os sexos, com idade igual ou superior a 60 anos, frequentadores de 58 ATIs representativas de todas as regiões programáticas dessa cidade. Os dados foram coletados utilizando-se questionário validado e analisados por meio de estatística descritiva. Resultado: Os resultados indicaram como motivos predominantes para justificar o ingresso dos idosos nas ATIs aqueles relacionados à saúde (Evitar problemas de saúde: 26%; O médico aconselhou: 16%), enquanto que os de permanência estão mais associados com questões sociais (Gosto do professor e Me faz sentir bem: 100%) e com a percepção dos benefícios do exercício físico (É uma atividade física saudável: 100%) Conclusão: Embora os motivos ligados à saúde figurem como importantes para os idosos ingressarem no programa, a sua permanência depende do gosto que eles têm pela atividade.

Abstract Objective: to verify the reasons behind the adherence (entry and permanence) of older adults to Gyms for Seniors (GFSs) in the city of Rio de Janeiro. Method: a total of 396 men and women over the age of 60 were interviewed for this study. The subjects frequented 58 different GFSs from all the administrative regions of the city of Rio de Janeiro. The data was collected with a validated questionnaire and analyzed through descriptive statistics. Results: the results indicated that the main reasons which explain the older population entering the GFSs are to do with health (Avoiding health problems: 26%; Doctor's orders: 16%) while their permanence is more associated with social factors (I like the teacher and It makes me feel good, both with 100%) and their perception of the benefits of exercise (It is a healthy physical activity: 100%). Conclusion: although health reasons are important for the older adults who enter the program, they mostly remain due to how much they like the activities.

Free exercise programs for the elderly: a systematic review on adherence and abandonment / Programas gratuitos de exercício físico para idosos: uma revisão sistemática sobre adesão e desistência

ABSTRACT The elderly population in Brazil is on the rise. As a consequence, there is an equal growth in the number of free public exercise programs directed to this group. Considering the benefits of exercising and the importance of adherence to this behavior so that the benefits can be perceived, we conducted a systematic review of the existing literature on the topic, aiming to identify the reasons that lead to adherence to free public exercise programs, as well as those that lead to abandoning the same programs. Searches were conducted on SciElo, SPORTdiscus and Pubmed databases, with search phrases in Portuguese and in English. After a triage, ten studies were analyzed regarding sample characteristics, program type, data collection method and the reasons behind the subjects' adherence and abandonment. We verified that elderly people tend to enter the projects for health-related reasons, but they remain due to other factors, such as socialization and the perception of the benefits brought on by exercising. As for abandonment, it occurs due to health problems, muscle pain due to exercising, dislike for the classes and family business.

RESUMO A população idosa cresce cada vez mais no Brasil. Como consequência, há um crescimento equivalente no número de programas públicos de exercícios físicos direcionados a esse grupo. Considerando os benefícios dos exercícios físicos e a importância de se aderir a esse comportamento para que os benefícios possam ser percebidos, foi realizada uma revisão sistemática da literatura existente sobre esse assunto com o objetivo de identificar os motivos que levam à adesão a programas gratuitos de exercícios, assim como aqueles que levam à desistência dos mesmos programas. Foram realizadas buscas nas bases de dados SciElo, SPORTdiscus e Pubmed, com frases de busca em português e em inglês. Após uma triagem, dez estudos foram analisados em relação às características das amostras, tipos de programas, método de coleta de dados e as razões por trás da adesão e da desistência dos sujeitos. Verificamos que as pessoas idosas tendem a entrar nos projetos por motivos relacionados à saúde, mas permanecem devido a outros fatores, como a socialização e a percepção dos benefícios provenientes da prática de exercícios. Quanto ao abandono, o mesmo ocorreu devido a problemas de saúde, dor muscular causada pelo exercício, o desgosto pelas aulas e questões relacionadas à família.

Do general practice management and/or team care arrangements reduce avoidable hospitalisations in Central and Eastern Sydney, Australia?

BACKGROUND: The number of people living with chronic health conditions is increasing in Australia. The Chronic Disease Management program was introduced to Medicare Benefits Schedule (MBS) to provide a more structured approach to managing patients with chronic conditions and complex care needs. The program supports General Practitioners (GP)s claiming for up to one general practice management plan (GPMP) and one team care arrangement (TCA) every year and the patient claiming for up to five private allied health visits. We describe the profile of participants who claimed for GPMPs and/or TCAs in Central and Eastern Sydney (CES) and explore if GPMPs and/or TCAs are associated with fewer emergency hospitalisations (EH)s or potentially preventable hospitalisations (PPH)s over the following 5 years. METHODS: This research used the CES Primary and Community Health Cohort/Linkage Resource (CES-P&CH) based on the 45 and Up Study to identify a community-dwelling population in the CES region. There were 30,645 participants recruited within the CES area at baseline. The CES-P&CH includes 45 and Up Study questionnaire data linked to MBS data for the period 2006-2014. It also includes data from the Admitted Patient Data Collection, Emergency Department Data Collection and Deaths Registry linked by the NSW Centre for Health Record Linkage. RESULTS: Within a two-year health service utilisation baseline period 22% (5771) of CES participants had at least one claim for a GPMP and/or TCA. Having at least one claim for a GPMP and/or TCA was closely related to the socio-demographic and health needs of participants with higher EHs and PPHs in the 5 years that followed. However, after controlling for confounding factors such as socio-demographic need, health risk, health status and health care utilization no significant difference was found between having claimed for a GPMP and/or TCA during the two-year health service utilisation baseline period and EHs or PPHs in the subsequent 5 years. CONCLUSIONS: The use of GPMPs and/or TCAs in the CES area appears well-targeted towards those with chronic and complex care needs. There was no evidence to suggest that the use of GPMPs and /or TCAs has prevented hospitalisations in the CES region.

Understanding the use and impact of allied health services for people with chronic health conditions in Central and Eastern Sydney, Australia: a five-year longitudinal analysis.

AIM: To describe the characteristics of people in Central and Eastern Sydney (CES), NSW, who had a General Practice Management Plan (GPMP) and claimed for at least one private allied health service item; and to examine if allied health service use results in less hospitalisations over a five-year period. BACKGROUND: The number of people living with chronic health conditions is increasing in Australia. The Chronic Disease Management programme was introduced to the Medicare Benefits Schedule (MBS) to provide a more structured approach to managing patients with chronic conditions and complex care needs. The programme supports general practitioners claiming up to one GPMP and one Team Care Arrangement every year, and the patient additionally claiming for up to five private allied health services visits. METHODS: A prospective longitudinal study was conducted. The sample consisted of 5771 participants in CES who had a GPMP within a two-year health service utilisation baseline period (2007-2009). The analysis used the 45 and Up Study questionnaire data linked to the MBS, hospitalisation, death and emergency department data for the period 2006-2014. FINDINGS: Of the eligible participants, 43% (2460) had at least one allied health service item claim in the subsequent 12 months. Allied health services were reported as physiotherapy, podiatry and other allied health services. The highest rates of allied health service use were among participants aged 85 years and over (49%). After controlling for confounding factors, a significant difference was found between having claimed for five or more physiotherapy services and emergency admissions (HR: 0.83; 95% CI: 0.72-0.95) and potentially preventable hospitalisations (HR: 0.79; 95% CI: 0.64-0.96) in the subsequent five years. Use of allied health service items was well targeted towards those with chronic and complex care needs, and use of physiotherapy services was associated with less avoidable hospitalisations.

Burden of Cancer Mortality in the Canadian Armed Forces, 1976-2012: A Retrospective Cohort Study.

BACKGROUND: Military personnel may have potential exposures to carcinogens during their military careers. However, the generalizability of causal evidence between occupational exposures and cancer outcomes in military personnel is limited. This study aims to describe the epidemiology cause-specific cancer mortality in still serving and released Canadian Armed Forces (CAF) personnel recruited between 1976 and 2012. METHODS: Data came from the Canadian Forces Cancer and Mortality Study II (CF CAMS II), a record-linkage study of approximately 228,685 CAF Regular Force personnel and Reservists. Sex-stratified standardized mortality ratios (SMR) were calculated for each neoplasm subcategory, with the Canadian general population (CGP) as the reference. RESULTS: Approximately 1,450 deaths were attributable to neoplasms. Cancer mortality was lower in both men and women with military service (SMR = 0.77 and 0.78, respectively) versus CGP. Females had a significantly lower risk of breast cancer. Males in the cohort had a significantly lower risk of lip, oral cavity and pharynx, digestive organs, respiratory and intrathoracic organs, bone and articular cartilage, and mesothelial and soft-tissue cancers. However, males also had a significantly increased risk for neoplasms of the central nervous system and lymphoid cells, as well as for certain specific cancer diagnoses. CONCLUSIONS: Current and former CAF personnel were at comparable, or lower risk than, the CGP for cancer-related deaths. However, there was an increased risk for certain neoplasm subcategories and specific cancers. IMPACT: These findings contribute to the limited body of evidence investigating the link between military service and cancer mortality.

Increasing use of general practice management and team care arrangements over time in New South Wales, Australia.

The number of older people living with chronic health conditions is increasing in Australia. The Chronic Disease Management (CDM) items program was introduced to the Medicare Benefits Schedule (MBS) to encourage a more structured approach to managing patients with chronic conditions. Initial uptake was slow and recent research has suggested that uptake is decreasing. This paper examines: person MBS CDM claims in NSW between 2006 and 2014 - using baseline survey data (2006-09) from the Sax Institute's 45 and Up Study linked to MBS and Death Registry data (2006-14) - and MBS CDM claims per 100000 population - using billing data sourced from the Medicare Australia Statistics website - to systematically examine any changes in uptake using a time-series analysis. After age adjustment, claims for initial plans increased from 11.3% in 2006 to 22.4% in 2014. Increases were also seen for allied health service claims (from 4.1% in 2006 to 20.8% in 2014) and for plan reviews (from 5.9% in 2006 to 16.0% in 2014). These increases were consistent with the MBS summary claims data. There is evidence that these plans are appropriately targeting those in most need; however, there is limited evidence of their effect. Claims for plan reviews, although increasing, are suboptimal and may indicate poor continuity of care.