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Currarino triad is a rare syndrome, with less than 250 cases reported, and it includes a combination of sacrococcigeal bony abnormalities, anorectal malformations and the presence of a presacral mass. Here we present a case of a 48-year-old male patient with history of severe chronic constipation, who was incidentally diagnosed with a presacral during imagiological investigation, which was histologically proven to be a neurendocrine tumor.

The aim of this study was to assess differences in etiology, comorbidities, echocardiographic parameters, and prognosis between men and women with significant tricuspid regurgitation (TR). Clinical and echocardiographic characteristics of 1569 patients (age 71 [62 to 78] years) at first diagnosis of significant TR were compared between men and women. Patients with congenital heart disease or previous tricuspid valve surgery were excluded. TR etiologies were defined as primary, left valvular disease related, left ventricular (LV) dysfunction related, pulmonary hypertension related, or isolated. The primary endpoint was all-cause mortality. Sex-specific differences in outcomes were compared in the total population and after propensity score matching. There were 798 (51%) women and 771 (49%) men in the study population. Women were diagnosed with significant TR at an older age compared with men (72 [62 to 79] years vs. 70 [61 to 77] years; p = 0.003). The TR etiology in women was more often left valvular disease related and isolated whereas men more often had LV dysfunction related TR. In the total population women had better 10-year survival compared with men (49% vs. 39%; p=0.001). After propensity score matching, the influence of sex on survival was neutralized (p = 0.228) but the TR etiologies remained significantly associated with all-cause mortality. Patients with left valvular disease or LV dysfunction related TR had lower survival compared with patients with primary TR (p = 0.004 and p = 0.019, respectively). In conclusion, long-term survival of patients with significant TR was similar between men and women after propensity score matching, while the etiology of TR remained significantly associated with all-cause mortality.

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant genetic disorder caused by germline mutation of the VHL gene. It is associated with multiple neoplasias including hemangioblastoma, clear cell renal cell carcinoma, pheochromocytoma, and neuroendocrine tumors. Ovarian tumors are extremely rare in this syndrome. We describe the case of a 16-yr-old girl with a previous diagnosis of bilateral pheochromocytoma and several pancreatic neuroendocrine tumors in VHL syndrome context. Follow-up abdominal-pelvic magnetic resonance imaging revealed a 33 mm, well-circumscribed nodule in the right ovary. The patient was submitted to laparoscopic right salpingo-oophorectomy. Microscopically, the tumor consisted of polygonal cells with abundant microvacuolized clear cytoplasm arranged in a solid pattern. The neoplastic cells were immunohistochemically positive for inhibin and calretinin. A diagnosis of ovarian steroid cell tumor was made. Only 4 cases with this association have been reported to date. Of the previously described cases, only one concerns a child; the others were all adult women. All of them had a previous diagnosis of VHL syndrome and presented with secondary amenorrhea and/or hirsutism due to testosterone-secreting ovarian steroid cell tumors. Although extremely rare, the association between VHL syndrome and ovarian steroid cell tumor has been reported, and our case suggests there is a link between the 2 entities.

Colonic intussusception is a rare cause of intestinal obstruction in adults and is caused by a malignant lesion in about 70% of cases. Early diagnosis and treatment are essential. We present a 64-year-old male patient with right colonic intussusception caused by a mixed adenoneuroendocrine carcinoma (MANEC), presenting as a giant pedunculated polyp (54 mm of largest diameter). The patient underwent right colectomy with primary anastomosis and adjuvant chemotherapy. The diagnosis of intussusception of the colon in adults is difficult because of its rarity and nonspecific clinical presentation. In this case, the cause was a rare histological type malignant tumor (MANEC).

There is increasing evidence that cardiorespiratory fitness (CRF) is an important health marker already in youth. This study aimed to determine the ability of five VO 2max equations to discriminate between low/high Metabolic Risk in 450 Portuguese adolescents aged 10–18. We measured waist and hip circumferences, fasting glucose, total cholesterol, HDL-cholesterol, LDL-cholesterol, triglycerides, and blood pressure. For each of these variables, a Z -score was computed. The HDL-cholesterol was multiplied by −1. A metabolic risk score was constructed by summing the Z scores of all individual risk factors. High risk was considered when the individual had ≥1 SD of this score. Cardiorespiratory fitness (CRF) was measured with the 20-m shuttle run test. We estimated VO 2max from the CRF tests using five equations. ROC analyses showed a significant discriminatory accuracy for the Matsuzaka and Barnett(a) equations in identifying the low/high metabolic risk in both genders (Matsuzaka girls: AUC = 0.654, 95%CI: 0.591–0.713, p 2max = 39.5 mL kg −1 min −1 ; boys: AUC = 0.648, 95%CI: 0.576–0.716, p 2max = 41.8 mL kg −1 min −1 ; Barnett(a) girls: AUC = 0.620, 95%CI: 0.557–0.681, p 2max = 46.4 mL kg −1 min −1 ; boys: AUC = 0.628, 95%CI: 0.555–0.697, p = 0.04, VO 2max = 42.6 mL kg −1 min −1 ), and the Ruiz equation in boys (AUC = 0.638, 95%CI: 0.565–0.706, p 2max = 47.1 mL kg −1 min −1 ). The VO 2max values found require further testing in other populations as well as in longitudinal studies; the identification of adolescents who have low CRF levels can help detect youth with an increased risk of metabolic disease.

The aim of this study was to verify the relation between body mass index (BMI) and sitting time in a sample of 4,091 Azorean men. BMI was calculated from self-reported weight and height. Total physical activity (PA) time and total sitting time were assessed with the IPAQ (short version). Linear Regression analysis showed that total sitting time (hours/day) was positively associated with BMI (B = 0.078, p < 0.001) after adjustments for age, meal frequency, alcohol and tobacco consumptions, island of residence, education level and total PA time. Although the cross sectional design precludes us from establishing causality, our findings emphasize the importance of reducing sedentary behavior to decrease the risk of obesity.

Background Physical inactivity is one of the four leading behavioural risk factors for non-communicable disease (NCD). Like tobacco control, increasing levels of health-enhancing physical activity (HEPA) will require a national policy framework providing direction and a clear set of actions. Despite frequent calls, there has been insufficient progress on policy development in the majority of countries around the world. This study sought and summarised national HEPA policy in seven European countries (Finland, Italy, the Netherlands, Norway, Portugal, Slovenia and Switzerland). Methods Data collection used a policy audit tool (PAT), a 27-item instrument structured into four sections. Results All countries reported some legislation or policy across the sectors of education, sport and health. Only some countries reported supportive policy in the transport and environment sectors. Five countries reported a stand-alone HEPA policy and six countries reported national recommendations. HEPA prevalence targets varied in magnitude and specificity and the presence of other relevant goals from different sectors highlighted the opportunity for joint action. Evaluation and the use of scientific evidence were endorsed but described as weak in practice. Only two countries reported a national multisector coordinating committee and most countries reported challenges with partnerships on different levels of policy implementation. Conclusions Bringing together the key components for success within a national HEPA policy framework is not simple. This in-depth policy audit and country comparison highlighted similarities and differences and revealed new opportunities for consideration by other countries. These examples can inform countries within and beyond Europe and guide the development of national HEPA policy within the NCD prevention agenda.

ObjectiveThere are no percentile curves for BMI, waist circumference (WC) or waist-to-height ratio (WHtR) available for Portuguese children and adolescents. The purpose of the present study was to develop age- and sex-specific BMI, WC and WHtR percentile curves for a representative sample of adolescents living in the Portuguese islands of Azores, one of the poorest regions of Europe, and to compare them with those from other countries.DesignCross-sectional school-based study. Weight, height and WC were objectively measured according to standard procedures. Smoothed percentile curves were estimated using Cole's LMS method.SettingAzores, Portugal.SubjectsProportionate stratified random sample of 1500 adolescents, aged 15–18 years.ResultsResults showed some sex differences in the shape of the BMI curves: in girls, the upper percentile values tend to decrease by the age of 16 and 17 years; whereas in boys, the upper percentiles tend to be flat between 15 and 16 years and then increase until the age of 18 years. In both sexes, the upper percentile values of both WC and WHtR decreased slightly by the age of 16 years and then increased steeply. In both sexes, the Azorean values for the 50th and 90th WC percentiles were higher than those reported for adolescents from the majority of other countries.ConclusionsThe reference curves presented herein provide baseline data for the long-term surveillance of Azorean adolescents, as well as for national and international comparisons.

Background In Portugal, there are several physical activity (PA) programmes for elderly people developed by the local government. The importance of these programmes has been increasing since the evidence has shown that this type of health promotion interventions may reduce the deleterious effects of the ageing process. However, no study has already identified the general characteristics of these programmes nor if they use any scheme to assess the quality of the service provided. A widely-used scheme is the EFQM Excellence Model, which will be in the core of our present work. Thus, the main aims of this preliminary study were 1) to identify the general characteristics of the PA programmes developed by the Portuguese Local Public Administration 2) to determine the extent of implementation of quality initiatives in these programmes. Methods Data were collected by an on-line questionnaire sent to all Continental Municipalities (n = 278). Categorical data were expressed as absolute counts and percentages. Continuous data were expressed as the mean and SD. An open-ended question was analysed using qualitative content analysis with QSR NVivo software. Associations between categorical variables were tested by the use of contingency tables and the calculation of chi-square tests. Significance level was set at p ≤ 0.05. Results Results showed: i) a total of 125 PA programmes were identified in the 18 districts of the Portugal mainland; ii) the main goal of the majority (95.2%) was the participants' health promotion; iii) different characteristics of the programmes were found according to different regions of the country; iv) certain characteristics of the programmes were associated to the existence of other features; v) only one PA programme developed quality initiatives. Conclusions In conclusion, although there are many PA programmes for elderly people spread throughout the country, aiming at improving the health of participants, the overwhelming majority does not adopt quality control initiatives. Considering that the quality of a service increases customer satisfaction, the continuous quality improvement of the PA programmes for elderly people should therefore be implemented since they can be useful and critical for elderly satisfaction and adherence.

Aim: The aims of this study were (1) to analyse the influence of cardiorespiratory fitness (CRF) and parental overweight status (POS) and socioeconomic status (SES) on abdominal obesity. Subjects and methods: This study was comprised of 779 adolescents (12‐18 years). Waist‐height ratio (WHtR), 20m shuttle-run test to ascertain CRF, POS according to World Health Organization recommendations and SES of parents using level of education were analysed. Results: Using WHtR, the prevalence of abdominal obesity was 21.3% (23.5% girls and 17.9% boys; p ¼ 0.062). Regardless of gender, participants who belonged to the WHtR risk group had significantly (p # 0.05) lower CRF scores than the WHtR nonrisk group; 84.4% of girls who belonged to the WHtR risk group had one or two overweight parents (p # 0.05). Boys with low CRF (OR: 6.43; CI: 3.33‐12.39) were more likely to belong to the WHtR risk group compared with their lean peers. Girls with low CRF (OR: 1.78; CI: 1.14‐2.78) and with at least one overweight parent (OR: 2.50; CI: 1.07‐5.85) or two overweight parents (OR: 4.90; CI: 2.08‐11.54) were associated with the risk of abdominal obesity. Conclusion: This study highlights the influence of adolescents’ family on abdominal obesity, especially in girls. Further, the data suggested that low CRF was a strong predictor of risk values of abdominal obesity in adolescence.

Background The prevalence of metabolic syndrome has increased over the last few decades in adolescents and has become an important health challenge worldwide. This study analyzed the relationships between metabolic risk factors (MRF) and physical activity (PA) and physical fitness (PF) in a sample of Azorean adolescents. Methods A cross-sectional school-based study was conducted on 417 adolescents (243 girls) aged 15-18 from the Azorean Islands, Portugal. Height, weight, waist circumference, fasting glucose, HDL-cholesterol, triglycerides, and blood pressure were measured. A sum of MRF was computed, and adolescents were classified into three groups: no MRF, one MRF and two or more MRF. PA was assessed by a sealed pedometer. PF was assessed using five tests from the Fitnessgram Test Battery. Dietary intake was obtained using a semi-quantitative food frequency questionnaire. Results Mean daily steps for girls and boys were 7427 ± 2725 and 7916 ± 3936, respectively. Fifty-nine percent of the adolescents showed at least one MRF and 57.6% were under the healthy zone in the 20 m Shuttle Run Test. Ordinal logistic regression analysis showed that after adjusting for sex, body mass index, socio-economic status and adherence to a Mediterranean diet, adolescents who were in the highest quartile of the pedometer step/counts (≥9423 steps/day) and those who achieved the healthy zone in five tests were less likely to have one or more MRF (OR = 0.56;95%CI:0.33-0.95; OR = 0.55;95%CI:0.31-0.98, respectively). Conclusions Daily step counts and PF levels were negatively associated with having one or more MRF among Azorean adolescents. Our findings emphasize the importance of promoting and increasing regular PA and PF to reduce the public health burden of chronic diseases associated with a sedentary lifestyle.

Metabolic syndrome, a predecessor of cardiovascular disease and type 2 diabetes, has become prevalent in adolescents. This study aimed to estimate the prevalence of metabolic syndrome and its components and to analyze the relationship between metabolic syndrome and overall physical fitness levels in a sample of Azorean adolescents.A cross-sectional school-based study, the Azorean Physical Activity and Health Study II, was conducted on 517 adolescents (297 girls, 220 boys) aged 15-18 years old from the Azorean Islands. Body height, weight, waist circumference, and arterial blood pressure were measured according to standards. Fasting intravenous blood samples were analyzed (high-density lipoprotein cholesterol, triglycerides, and glucose). Physical fitness was assessed using five tests from Fitnessgram Test Battery 8.0: Curl-up, push-up, trunk lift, pacer, and sit-and-reach. Adolescents were then classified as being in the healthy zone or above or under the healthy zone. Metabolic syndrome was defined according to the 2007 International Diabetes Federation‘s guidelines for adolescents.The prevalence of metabolic syndrome was 5% (4.7% in girls and 5.5% in boys, P0.05). Waist circumference was the most prevalent component (32.9%), and hypertriglyceridemia the least (4.4%). Logistic regression analysis showed that after adjusting for pubertal stage and socioeconomic status, unfit adolescents (healthy zone criteria inor=2 tests) were more likely [odds ratio (OR) = 3.414; 95% confidence interval (CI), 1.150-10.129] to be classified as having metabolic syndrome.The prevalence of metabolic syndrome is high in Azorean adolescents. Unfit adolescents were more likely to have metabolic syndrome than fit adolescents. Improving overall physical fitness levels and abdominal obesity reduction may be important strategies in overcoming this public health problem and its consequences.

Background In the past years, there has been a growing concern in designing physical activity (PA) programmes for elderly people, because evidence suggests that such health promotion interventions may reduce the deleterious effects of the ageing process. Quality is an important issue when designing a PA programme for older people. Some studies support the Excellence Model of the European Foundation for Quality Management (EFQM) as an operational framework for evaluating the quality of an organization. Within this context, the aim of this study was to characterize the quality management models of the PA programmes developed by Portuguese Local Administration to enhance quality of life for elderly people, according to the criteria of the EFQM Excellence Model. Methods A methodological triangulation was conducted in 26 PA programmes using questionnaire surveys, semi-structured interviews and document analysis. We used standard approaches to the statistical analysis of data including frequencies and percentages for the categorical data. Results Results showed that Processes (65,38%), Leadership (61,03%), Customer results (58,46) and People (51,28%) had high percentage occurrences of quality practices. In contrast, Partnerships and resources (45,77%), People results (41,03%), Policy and strategy (37,91%), Key performance results (19,23%) and Society results (19,23%) had lower percentage occurrences. Conclusions Our findings suggest that although there are some good practices in PA programmes, there are still relevant areas that require improvement.

Background Aging is one of the most important and obvious phenomenon observed in our society. In the past years, there has been a growing concern in designing physical activity (PA) programmes for elderly people, because evidence suggests that such health promotion interventions may reduce the deleterious effects of the ageing process. Accordingly, a growing body of literature points to the importance of a sound approach to planning and evaluation in order to improve the quality of PA programmes. However, while numerous PA programmes have been designed for the elderly in recent years, their evaluation has been scarce. Quality management processes and tools provide a practical way for organisations to assess, identify and shed light on the areas requiring improvement. The Quality Self-assessment Tool for Exercise Programmes for Seniors (Q-STEPS) seems to provide a framework tailored to evaluate PA programmes for the elderly. Findings The primary purpose of this study is 1) to determine feasibility, acceptability and usability of the Q-STEPS. Secondary purposes of the study are: 2) to examine the quality of the PA programmes for elderly people developed by the Portuguese Local Administration over a three-year period of self-assessments in terms of: a) Enabler domains (Leadership, Policy and Strategy, People, Partnership and Resources, Processes); b) Result domains (Customer Results, People Results, Society Results and Key Performance Results); 3) to estimate the association between the use of Q-STEPS and some indicators relating to the elderly participants, during the three self-assessments, such as: attendance rates, physical fitness, health-related quality of life and the elderly’s perceived quality of the programme. The study will be conducted in PA programmes for elderly adults from mainland Portuguese municipalities over a three-year period. The project will adopt a participative quality improvement approach that features annual learning cycles of: 1) self-assessment with the Q-STEPS; 2) feedback to and interpretation of results involving programme’s staff; 3) action planning to achieve system changes; 4) implementation of strategies for change; and 5) review process through further self-assessment. The study will collect a range of process and outcome data that will be used to achieve the research aims. Discussion It is our understanding that the results of the Q-STEPS study will contribute directly to the evidence based on effectiveness of continuous quality improvement approaches, in order to improve customer satisfaction and adherence to PA programmes targeting the ageing population. This comprehensive evaluation will also add significant new knowledge regarding the characteristics associated with a sustainable public service.