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Synthetic progestins are micropollutants of special concern, due to their growing use in human and veterinary therapies and their risks to aquatic life. Currently, there is a lack of environmental information on these compounds, worldwide. The main objective of this work was to characterize the levels of the most consumed progestins in Portugal. For that, Ria de Aveiro, Tagus estuary and Ria Formosa were sampled in a temporal perspective to evaluate levels of drospirenone (DRO), desogestrel (DSG), gestodene (GST) and levonorgestrel (LNG). Drospirenone and desogestrel were the most abundant progestins. In the North of Portugal, DSG was the most abundant (Aveiro: 193.9 ng L -1 in summer), while DRO was more representative in the South (Tagus: 178.9 ng L -1 ; Formosa: 125.7 ng L -1 ) and also in summer. These spatial differences can be associated with the hydrodynamics of each estuarine system as well as the distinct population and tourist levels associated with each site., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Patricia Cardoso reports financial support was provided by Foundation for Science and Technology. Hugo Morais reports financial support was provided by Foundation for Science and Technology. Patricia Cardoso reports financial support was provided by Ocean3R (NORTE-01-0145-FEDER-000064)., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

This article aims to study the prevalence of coronary artery calcification and associated factors in a multiethnic population in Angola., Methods: A descriptive, cross-sectional observational study was carried out in a private clinic in Angola. For this purpose, information was collected from sociodemographic and biological data. The selected variables were; history of arterial hypertension, diabetes mellitus, smoking (current and past), alcohol consumption, family history of coronary disease, and coronary calcium score. Independent Mann-Whitney test, Student's t -test and chi-square test were used as appropriate., Results: The sample consisted of 211 individuals: 156(73.9%) of black race, 37(17.4%) of mixed race and 18(8.4%) of Caucasian race. 126(59.7%) were male. The average age was 56.7 ± 9.3 years. Of the total sample, 158 (74.9%) had a history of hypertension, 50 (23.7%) of diabetes mellitus, and 138 (65.4%) of dyslipidemia. Of the total number of individuals, 21(10.0%) were smokers and 38(18.0%) were ex-smokers, 137 (64.9%) were social drinkers and 44(20.9%) were obese. A significant association was found between calcification of the coronary arteries and aging (p <.001), Caucasian race (p =.037), and a history of diabetes mellitus, dyslipidemia and smoking (p <.001, p <.001, p =.012, respectively). Black race and female gender are associated with a lower risk of coronary artery calcification (p =.034 and p =.011, respectively)., Conclusion: The present results support the notion that there are racial and ethnic differences in the prevalence of coronary calcification., Competing Interests: The authors declare no conflicts of interest and no specific funding sources for this work., (Copyright ©2023 The Author(s).)

The antidiabetic drug Metformin (MET), one of the most prevalent pharmaceuticals in the environment, is currently detected in surface waters in the range of ng/L to low μg/L. As current knowledge regarding the long-term effects of environmentally relevant concentrations of MET in nontarget organisms is limited, the present study aimed at investigating the generational effects of MET, in concentrations ranging from 390 to 14 423 ng/L in the model organism Danio rerio (up to 9 mpf), including the effects on its nonexposed offspring (until 60 dpf). We integrate several apical end points, i.e., embryonic development, survival, growth, and reproduction, with qRT-PCR and RNA-seq analyses to provide additional insights into the mode of action of MET. Reproductive-related parameters in the first generation were particularly sensitive to MET. MET parental exposure impacted critical molecular processes involved in the metabolism of zebrafish males, which in turn affected steroid hormone biosynthesis and upregulated male vtg1 expression by 99.78- to 155.47-fold at 390 and 14 432 MET treatment, respectively, pointing to an estrogenic effect. These findings can potentially explain the significant decrease in the fertilization rate and the increase of unactivated eggs. Nonexposed offspring was also affected by parental MET exposure, impacting its survival and growth. Altogether, these results suggest that MET, at environmentally relevant concentrations, severely affects several biological processes in zebrafish, supporting the urgent need to revise the proposed Predicted No-Effect Concentration (PNEC) and the Environmental Quality Standard (EQS) for MET.

Introduction: Left ventricular thrombus commonly occurs as a complication of acute anterior myocardial infarction and nonischemic cardiomyopathies with severe left ventricular systolic dysfunction. Its frequency is still high despite medical advances. Current guidelines recommend the use of vitamin k antagonists as first-line therapy, however, the off-label use of direct oral anticoagulants is becoming more frequent and attractive, given the better pharmacological and clinical profile, with the improvement of the patient's quality of life., Aim: To provide an update on the currently existing evidence regarding the outcomes of efficacy and safety of direct oral anticoagulants (DOACs) as first-line therapy in left ventricular thrombus, in comparison to vitamin K antagonists (VKAs)., Methods: A systematic review and meta-analysis of studies on the effects of direct oral anticoagulants versus vitamin K antagonists on left ventricular thrombi and on the results was performed., Results: Fourteen studies were included in the meta-analysis, with a total of 2498 patients (n=631 direct oral anticoagulants and n=1867 for VKAs). No significant differences were found in efficacy and safety outcomes (odds ratio (OR) 0.86; 95% confidence interval (CI), 0.55-1.33; p=0.50; I 2 =32%) and (OR 1.0; 95% CI, 0.78-1.30; p=0.93; I 2 =2%) respectively. No difference was noted in all-cause mortality (OR 0.92; 95% CI, 0.58-1.45; p=0.74; I 2 =0%). Thrombus resolution was observed in 288/416 in direct oral anticoagulants vs. 732/1085 patients treated with VKAs (OR 1.14; 95% CI, 0.77-1.66; p=0.50; I 2 =33%)., Conclusions: The findings of this meta-analysis suggest the potential utility of DOACs as a first-line strategy in patients with left ventricular thrombus., (Copyright © 2022 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

The present article describes the prevalence of coronary artery anomalies (CAAs) in patients undergoing coronary CT angiography at a private clinic in Luanda, Angola, and the clinical and angiotomographic characteristics of the two patients with CAAs. These anomalies are uncommon and constitute a significant challenge in resource-poor countries., Competing Interests: None., (© 2022 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Metformin (MET), an anti-diabetic pharmaceutical of large-scale consumption, is increasingly detected in surface waters. However, current knowledge on the long-term effects of MET on non-target organisms is limited. The present study aimed to investigate the effects of MET in the model freshwater teleost Danio rerio, following a full life-cycle exposure to environmentally relevant concentrations (390 to 14 423 ng/L). Considering that the mode of action (MoA) of MET on non-target organisms remains underexplored and that MET may act through similar human pathways, i.e., lipid and energy metabolisms, biochemical markers were used to determine cholesterol and triglycerides levels, as well as mitochondrial complex I activity in zebrafish liver. Also, the hepatosomatic index as an indication of metabolic disruption, and the expression levels of genes involved in MET's putative MoA, i.e. acaca, acadm, cox5aa, idh3a, hmgcra, prkaa1, were determined, the last by qRT-PCR. A screening of mRNA transcripts, associated with lipid and energy metabolisms, and other signaling pathways potentially involved in MET-induced toxicity were also assessed using an exploratory RNA-seq analysis. The findings here reported indicate that MET significantly disrupted critical biochemical and molecular processes involved in zebrafish metabolism, such as cholesterol and fatty acid biosynthesis, mitochondrial electron transport chain and tricarboxylic acid cycle, concomitantly to changes on the hepatosomatic index. Likewise, MET impacted other relevant pathways mainly associated with cell cycle, DNA repair and steroid hormone biosynthesis, here reported for the first time in a non-target aquatic organism. Non-monotonic dose response curves were frequently detected in biochemical and qRT-PCR data, with higher effects observed at 390 and 2 929 ng/L MET treatments. Collectively, the results suggest that environmentally relevant concentrations of MET severely disrupt D. rerio metabolism and other important biological processes, supporting the need to revise the proposed environmental quality standard (EQS) and predicted no-effect concentration (PNEC) for MET., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Teresa Neuparth reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia and INTERREG. Miguel Santos reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Susana Barros reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Marlene Pinheiro reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Hugo Morais reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Nelson Alves reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Ana M. Coimbra reports financial support was provided by FCT - Fundação para a Ciência e a Tecnologia. Jose Benito Quintana reports was provided by Xunta de Galicia. Jose Benito Quintana reports financial support was provided by Agencia Estatal de Investigación., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Introduction: Studies on the normal echocardiographic reference values in Africans are limited., Objectives: This study aims to establish the normal left ventricular echocardiographic parameters for adult Angolans, stratified by gender and age., Methods: A cross-sectional study was performed involving healthy adults attending a diagnostic center in Luanda, Angola. The two-dimensional transthoracic echocardiography was performed according to the Guidelines of the American Society of Echocardiography and the European Association of Cardiovascular Imaging., Results: A total of 103 men (47.5%) (mean age: 39.5 ± 10,8) and 114 women (52.5%) (mean age: 43.0 ± 12,5 years) were included. Compared to men, women were older ( p  = 0.025) and had a significantly smaller body surface area (BSA) ( p  < 0.001). Left ventricular wall thickness, left ventricular end-diastolic dimension and volume (LVEDV), left ventricular mass (LVM) and LVM indexed to BSA (LVMi) were significantly lower in women ( p  < 0.005). LVEDD indexed to BSA and left ventricular ejection fraction (LVEF) were significantly higher in women than in men ( p  = 0.007), ( p  = 0.01), respectively. Mitral annular plane systolic excursion, LVEF by strain, and global longitudinal strain showed no gender differences. Posterior wall thickness showed a statistically significant increase in the older groups ( p  = 0.043). The VST, relative wall thickness, LVM, and LVMi showed no significant differences between age categories. In turn, the shortening fraction and the ejection fraction increase with age., Conclusion: For a more sensitive morphological and functional assessment of the left ventricle, it is necessary to take into account the gender and age of the individual., Competing Interests: The authors declare no conflicts of interest and no specific funding sources for this work., (Copyright ©2022 The Author(s).)

Introduction: Studies about the reference values of conventional and tissue Doppler echocardiography in African populations are limited., Objectives: To evaluate the effect of aging and gender in left ventricular diastolic function assessed by conventional and tissue Doppler echocardiography in healthy Angolans., Methods: Cross-sectional study involving healthy adult participants attending a diagnostic center in Luanda, Angola. Two-dimensional transthoracic B-mode and Doppler echocardiography were performed according to the Guidelines of the American Society of Echocardiography and the European Association of Cardiovascular Imaging., Results: A total of 103 men (47.5%) (mean age: 39.5 ± 10.8) and 114 women (52.5%) (mean age: 43.0 ± 12.5 years) were included. Women were older (p = 0.025) and showed smaller body surface area (p < 0.001) than men. Mitral E velocity and E/e' ratio were higher in women than in men. While DT of mitral E velocity was longer in men than in women (p = 0.041), E velocity, E/A ratio, and average e' velocity showed a negative significant correlation with increasing age (r - 0.33; p ≤ 0.001; r - 0.48; p ≤ 0.001 and r - 0.41; p ≤ 0.0001, respectively). Conversely, A wave velocity showed a significant positive correlation with age (r 0.47; p < 0.001)., Conclusions: In a healthy Angolan adult population, the variables of left ventricular diastolic function declined with age, reflecting a normal aging process. This must be taken into account when assessing diastolic function at different age categories., (© 2021 Wiley Periodicals LLC.)

Methicillin-resistant Staphylococcus aureus (MRSA) has long been known as a major cause of hospital-acquired (HA-MRSA) infections worldwide. For the past twenty years, an increasing number of studies have described its emergence in the community as well. In Portugal, a country with a high-prevalence of HA-MRSA, there are only limited data available on the epidemiology of MRSA in the community. We studied the prevalence of S. aureus and MRSA colonization among healthy adults in Portugal. Between February 2015 and December 2016, a longitudinal study was conducted in which 87 adults aged 25-50 years old were followed for six months. For each participant nasopharyngeal, oropharyngeal and saliva samples were obtained monthly and, in some cases, weekly. A total of 1,578 samples (n = 526 for each sampling site) were examined for the presence of S. aureus and MRSA by classical culture-based methods. Fifty-seven adults (65.5%) carried S. aureus at least once during the six months period of the study: 19.5% were persistent S. aureus carriers and 46.0% were intermittent carriers. Carriage rates per sampling site were 20.5% in nasopharynx, 18.3% in oropharynx, and 13.5% in saliva. Simultaneous screening of the three sampling sites increased detection of S. aureus, which overall occurred in 34.4% of the 526 sampling time-points. No MRSA were isolated. In conclusion, this study adds novel information about the MRSA scenario in the Portuguese community. Our results indicate that, in Portugal, MRSA does not seem to circulate among healthy adults without risk factors and therefore this age group does not constitute, at the current time, a reservoir of MRSA in the community., Competing Interests: This work was partially supported by an Investigator-Initiated Research Grant from Pfizer to RSL. Other authors declare no competing interests. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

miRNA(miR)-124 is an important regulator of neurogenesis, but its upregulation in SOD1G93A motor neurons (mSOD1 MNs) was shown to associate with neurodegeneration and microglia activation. We used pre-miR-124 in wild-type (WT) MNs and anti-miR-124 in mSOD1 MNs to characterize the miR-124 pathological role. miR-124 overexpression in WT MNs produced a miRNA profile like that of mSOD1 MNs (high miR-125b; low miR-146a and miR-21), and similarly led to early apoptosis. Alterations in mSOD1 MNs were abrogated with anti-miR-124 and changes in their miRNAs mostly recapitulated by their secretome. Normalization of miR-124 levels in mSOD1 MNs prevented the dysregulation of neurite network, mitochondria dynamics, axonal transport, and synaptic signaling. Same alterations were observed in WT MNs after pre-miR-124 transfection. Secretome from mSOD1 MNs triggered spinal microglia activation, which was unno-ticed with that from anti-miR-124-modulated cells. Secretome from such modulated MNs, when added to SC organotypic cultures from mSOD1 mice in the early symptomatic stage, also coun-teracted the pathology associated to GFAP decrease, PSD-95 and CX3CL1-CX3CR1 signaling im-pairment, neuro-immune homeostatic imbalance, and enhanced miR-124 expression levels. Data suggest that miR-124 is implicated in MN degeneration and paracrine-mediated pathogenicity. We propose miR-124 as a new therapeutic target and a promising ALS biomarker in patient sub-populations.

Background: Endoscopic sleeve gastroplasty (ESG) is an option for patients with Class I and II obesity or patients who refuse to undergo a laparoscopic bariatric surgery. The aims of this study are as follows: (1) to demonstrate a short-term outcome after primary ESG and (2) to compare the effectiveness of weight loss between Class I and Class II obesity patients., Methods: Patients undergoing ESG at four bariatric centers in Brazil between April 1, 2017 and December 31, 2018 were prospectively enrolled in the study (BMI 30.0-39.9 kg/m 2 ). ESG was performed using Overstitch (Apollo Endosurgery, Austin, TX). Descriptive analysis, t test, Chi-square test, and Mann-Whitney test were used to present the results., Results: A total of 233 patients underwent primary ESG. The mean age and BMI of the patients were 41.1 years and 34.7 kg/m 2 , respectively. Following ESG, the mean percentage of total weight loss (TWL) was 17.1% at 6 months and 19.7% at 12 months. Percentage of excess BMI loss (EBMIL) was 47.3% at 6 months and 54.8% at 12 months after ESG. The mean EBMIL was significantly greater among patients with Class I obesity than those with Class II obesity at 6 (51.1% vs. 43.7%) and 12 months (60.2% vs. 49.2%). One patient experienced bleeding during the procedure that was managed with sclerotherapy., Conclusion: Short-term results suggest that ESG is a safe and effective option for patients with Class I and II obesity.

Introduction: Single ventricle physiology management is challenging, especially in low-income countries., Objective: To report the palliation outcomes of single ventricle patients in a developing African country., Methods: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock-Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure)., Results: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival., Conclusion: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.

The present study evaluated the effects of acute treatment with silymarin, an extract that is obtained from Silybum marianum, on angiogenesis, oxidative stress, and inflammation in normoglycemic and diabetic mice. Diabetes was induced by streptozotocin (80 mg/kg, intraperitoneal) in male Swiss mice, 6 weeks of age. A polyether-polyurethane sponge was surgically implanted in the back of the mice as a model of healing in both diabetic and normoglycemic animals that were treated with oral silymarin or water for 10 days. The pancreas, liver, kidneys, blood, and sponges were collected and analyzed. Diabetes led to impairments of antioxidant defenses, reflected by a reduction of pancreatic superoxide dismutase and hepatic and renal catalase and an increase in pancreatic lipoperoxidation. An inflammatory process was observed in diabetic mice, reflected by an increase in pancreatic tumor necrosis factor α (TNF-α) and the infiltration of inflammatory cells in islets. The number of vessels was lower in the implanted sponges in diabetic mice. Silymarin treatment attenuated this damage, restoring antioxidant enzymes and reducing pancreatic TNF-α and inflammatory infiltration. However, silymarin treatment did not restore angiogenesis or glycemia. In conclusion, treatment with silymarin red uced oxidative stress and inflammation that were induced in the model of streptozotocin-induced diabetes in several organs, without apparent toxicity. Silymarin may be a promising drug for controlling diabetic complications., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

To effectively address the needs of youth who perpetrate sexual violence and reduce rates of recidivism, a better understanding of the mechanisms of juvenile sexual offending is needed. Current literature identifies various factors that are believed to put youth at risk for sexual offending, two of which are empathy deficits and childhood sexual abuse (CSA). The extent to which empathy deficits contribute to juvenile sexual offending, however, is often debated, though studies have not yet explored a neurobehavioral model of this mechanism. This pilot study used functional magnetic resonance imaging (fMRI) to explore the neural correlates of empathy in juveniles who sexually offend (JSOs), and the possible role of CSA. A total of 38 males (ages 12-20) were enrolled, including 11 healthy control subjects and 27 JSOs, of which, 11 had a history of CSA. Participants underwent clinical assessment and completed an empathy task during fMRI. Using both whole-brain and region-of-interest analysis, results of the fMRI data showed no statistical differences in engagement of brain regions associated with empathy between controls and all JSOs. There were also no significant differences between JSOs with and without a history of CSA. These null findings pose implications for guiding future research studies with larger samples and more statistical power, and may support the need to further explore empathy-related explanatory models and interventions for JSOs. Neuroimaging may demonstrate to be a useful tool to identify individualized risk factors and aid in tailoring interventions for this population.

A subannular left ventricular aneurysm is very rare, and is mostly considered to be a congenital anomaly. A subannular left ventricular aneurysm is classified based on the type of its own orifice-submitral or subaortic. Subaortic left ventricular aneurysm occurs less frequently compared with a submitral type of subannular aneurysm. We hereby describe a rare case of a huge bilobed subaortic aneurysm, in which the orifice was located just below the left coronary cusp diagnosed with multimodality imaging in a child., (© 2017, Wiley Periodicals, Inc.)

Background: Until recently, intravenous thrombolysis was the only reperfusion therapy with proven efficacy in patients with acute ischemic stroke. However, this treatment option has low recanalization rates in large-vessel occlusions. The search for additional treatments continued until 5 randomized trials (MR CLEAN, ESCAPE, EXTEND-IA, SWIFT PRIME, and REVASCAT) revealed the superiority of mechanical thrombectomy for anterior circulation large-vessel occlusion. After 1 year of performing thrombectomy with stent retrievers in our tertiary hospital, we intended to answer the question: is it possible to achieve similar results in a "real-world" setting?, Methods: We analyzed data from our prospective observational registry, compared it with the trials aforementioned, and concluded that the answer is affirmative., Results: Our study population of 77 patients, with a mean age of 68,2 years and 48,1% men, is comparable with these trials in much of selection criteria, baseline characteristics, and rate of previous intravenous thrombolysis (72,7%). Recovery of functional independence at 90 days was achieved in almost two thirds of patients, similarly to the referred trials. We devoted special emphasis on fast recanalization, keeping a simple image selection protocol (based on non-enhanced and computed tomography angiography) and not waiting for clinical response to thrombolysis in patients eligible for mechanical thrombectomy. We emphasize a successful recanalization rate of 87% and only 2,6% symptomatic intracranial hemorrhage., Conclusion: In summary, mechanical thrombectomy seems to be a safe and effective treatment option in a "real-world" scenario, with results similar to those of the recent randomized controlled trials., (Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Objectives: Identifying the factors affecting the outcome of patients with Fournier's Gangrene and assaying the accuracy of the Fournier Gangrene Severity Index (FGIS), the Uludag score (UdS), affected Body Surface Area (BSA) and the Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) model as prognostic tools., Materials and Methods: Retrospective study involving all patients with Fournier's gangrene treated in our Hospital between January 2008 and December 2015. The epidemiological, clinical, biochemical and management data of these patients was obtained and analyzed., Results: The series includes 19 patients, 14 male and 5 female, with a median age of 70 (62; 78,5) years. The mortality rate was 21%. From the data analyzed, only the affected BSA (BSA>3.25%) was associated with mortality (p = 0.016). None of the established scores (FGSI; UdS; LRINEC) proved to be a useful tool for predicting mortality. The combination of affected BSA and FSGI (FGSI≥9 or BSA>3.25%), (p = 0.004) and the combination of the affected BSA and the LRINEC model (LRICEC≥8 and BSA>3.25%), (p = 0.004) led to a major improvement in these scores., Conclusions: Affected BSA is a useful prognostic factor in Fournier's gangrene. The existing prognostic scores can be improved with the introduction of this factor.

Chlorpyrifos is a pesticide, member of the organophosphate class, widely used in several countries to manage insect pests on many agricultural crops. Currently, chlorpyrifos health risks are being reevaluated due to possible adverse effects, especially on the central nervous system. The aim of this study was to investigate the possible action of this pesticide on the behaviors related to anxiety and depression of offspring rats exposed during pregnancy. Wistar rats were treated orally with chlorpyrifos (0.01, 0.1, 1 and 10mg/kg/day) on gestational days 14-20. Male offspring behavior was evaluated on post-natal days 21 and 70 by the elevated plus-maze test, open field test and forced swimming test. The results demonstrated that exposure to 0.1, 1 or 10mg/kg/day of chlorpyrifos could induce anxiogenic-like, but not depressive-like behavior at post-natal day 21, without causing fetal toxicity. This effect was reversed on post-natal day 70., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Disulfiram (tetraethylthiuram disulfide) has been used for the treatment of alcohol dependence. An axonal sensory-motor polyneuropathy with involvement of cranial pairs due to disulfiram is exceedingly rare. The authors report a unique case of an extremely severe axonal polyneuropathy involving cranial nerves that developed within weeks after a regular dosage of 500mg/day disulfiram. To the authors best knowledge, such a severe and rapidly-progressive course has never been described with disulfiram dosages of only 500mg/day., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Introduction: In this study we estimated the prevalence, incidence, and mortality of myasthenia gravis (MG) in northern Portugal and characterized the clinical features of the patients identified., Methods: We used 2 data sources: clinical records from the hospitals and pyridostigmine prescription registers., Results: On December 31, 2013, we estimated a point prevalence of 111.7 patients per million population. The highest prevalence was observed in the group >65 years of age, especially in men (288.1 per million). During 2013, we estimated an incidence rate of 6.3 per million per year. Among women, the incidence rate was highest in the 15-49-year age group; in men, incidence increased with age up to 22.1 per million in those >65 years old. The MG-related mortality rate was 0.5 per million., Conclusions: These figures are in keeping with similar studies and emphasize the importance of diagnosis and management of MG in elderly populations. Muscle Nerve 54: 413-421, 2016., (© 2016 Wiley Periodicals, Inc.)

Background: We report a surgical series of submitral aneurysm in children., Methods: Between March 2011 and December 2015, eight consecutive patients less than 18 years old with submitral aneurysm underwent surgical correction., Results: Six patients were female, the mean age was 7 ± 3.8 years old, and mean weight was 21.4 kg. Six patients were in NYHA functional class III or IV. Six patients underwent repair via a transatrial approach, another with a transatrial combined with transaneurysmal approach, and another with a transventricular approach. There were no in-hospital deaths but one 30-day mortality. One patient required reoperation. Two patients required mitral valve replacement. At discharge, one patient had severe and another had moderate mitral regurgitation. The mean follow-up time was 26.4 months and five patients were alive. No reintervention was required., Conclusions: Submitral aneurysm is not restricted to adults. Heart failure is the commonest clinical presentation in the pediatric age. The transatrial approach is feasible, safe, and associated with good short-term results. The mitral valve can be preserved in the majority of cases., (© 2016 Wiley Periodicals, Inc.)

The increase of distributed energy resources, mainly based on renewable sources, requires new solutions that are able to deal with this type of resources' particular characteristics (namely, the renewable energy sources intermittent nature). The smart grid concept is increasing its consensus as the most suitable solution to facilitate the small players' participation in electric power negotiations while improving energy efficiency. The opportunity for players' participation in multiple energy negotiation environments (smart grid negotiation in addition to the already implemented market types, such as day-ahead spot markets, balancing markets, intraday negotiations, bilateral contracts, forward and futures negotiations, and among other) requires players to take suitable decisions on whether to, and how to participate in each market type. This paper proposes a portfolio optimization methodology, which provides the best investment profile for a market player, considering different market opportunities. The amount of power that each supported player should negotiate in each available market type in order to maximize its profits, considers the prices that are expected to be achieved in each market, in different contexts. The price forecasts are performed using artificial neural networks, providing a specific database with the expected prices in the different market types, at each time. This database is then used as input by an evolutionary particle swarm optimization process, which originates the most advantage participation portfolio for the market player. The proposed approach is tested and validated with simulations performed in multiagent simulator of competitive electricity markets, using real electricity markets data from the Iberian operator-MIBEL.

We report an incidental finding of pseudoaneurysm in a 10-month-old boy with tetralogy of Fallot and Down syndrome who had undergone placement of a modified Blalock-Taussig shunt at age four months. Computed tomography was a determinant exam for better assessment. The lesion was successfully resected with concomitant complete repair of tetralogy of Fallot in a single-stage. The child was asymptomatic at fourth month follow-up., (© The Author(s) 2016.)

Background: CADe and CADx systems for the detection and diagnosis of lung cancer have been important areas of research in recent decades. However, these areas are being worked on separately. CADe systems do not present the radiological characteristics of tumors, and CADx systems do not detect nodules and do not have good levels of automation. As a result, these systems are not yet widely used in clinical settings., Methods: The purpose of this article is to develop a new system for detection and diagnosis of pulmonary nodules on CT images, grouping them into a single system for the identification and characterization of the nodules to improve the level of automation. The article also presents as contributions: the use of Watershed and Histogram of oriented Gradients (HOG) techniques for distinguishing the possible nodules from other structures and feature extraction for pulmonary nodules, respectively. For the diagnosis, it is based on the likelihood of malignancy allowing more aid in the decision making by the radiologists. A rule-based classifier and Support Vector Machine (SVM) have been used to eliminate false positives., Results: The database used in this research consisted of 420 cases obtained randomly from LIDC-IDRI. The segmentation method achieved an accuracy of 97 % and the detection system showed a sensitivity of 94.4 % with 7.04 false positives per case. Different types of nodules (isolated, juxtapleural, juxtavascular and ground-glass) with diameters between 3 mm and 30 mm have been detected. For the diagnosis of malignancy our system presented ROC curves with areas of: 0.91 for nodules highly unlikely of being malignant, 0.80 for nodules moderately unlikely of being malignant, 0.72 for nodules with indeterminate malignancy, 0.67 for nodules moderately suspicious of being malignant and 0.83 for nodules highly suspicious of being malignant., Conclusions: From our preliminary results, we believe that our system is promising for clinical applications assisting radiologists in the detection and diagnosis of lung cancer.

The management of cluster headache (CH) may be challenging. We report a 50-year-old male with recurrent attacks of dull and severe unilateral periorbital pain, lasting 30-45 minutes, twice a day, exclusively during sleep, and accompanied by ipsilateral rhinorrhea and lacrimation. The pain switched sides within every attack. CH treatment was initiated but the patient maintained recurrence rates compatible with chronic CH, even after increasing verapamil to 460 mg/day. Afterwards we decided to add lithium (800 mg/day). With this treatment the severity and recurrence of CH substantially decreased, despite the patient's autonomous decision to take lithium only during the acute phase of the cluster. The exclusively alternating location and the excellent response to short cycles of lithium represent two unique features of CH., Competing Interests: The authors declare that there is no conflict of interests.

Background: Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta., Objective: To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal-Angola collaboration., Methods: Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension., Results: There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required., Conclusion: Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results., (© The Author(s) 2015.)

Introduction and Objective: Ebstein's anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola., Methods: A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects., Results: Of the 1362 patients studied, eight (0.6%) had Ebstein's anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I., Conclusion: The frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results., (Copyright © 2015 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Background: Urinary tract infection caused by Escherichia coli is a frequently observed condition both in humans and animals. Uropathogenic E. coli (UPEC) has been shown to have a pathogenicity island that enables them to infect the urinary tract. Because there is little information about the presence of UPEC-associated virulent genes in animal isolates this work was carried out with the intent to enhance the understanding about the strains of E.coli that cause infections in animals., Results: We screened 21 E. coli strains isolated causing urinary tract infection in domestic animals. Primers were designed to amplify urinary infection-associated genes. Nine genes, pap A, tcp C , fyu A, tpb A, Lma, hyl A, pic U, ton B, and flic C were then amplified and sequenced. Different from the human isolate CFT073, all the animals E. coli lack some of the pathogenesis-associated genes. Genes encoding for proteins used to scavenge iron appear not to be so necessary during animal infections as they are in human infection. In further investigation of phenotypic properties, it was observed that animal UPECs have significantly more impaired ability to form biofilms than human UPEC strain., Conclusions: This study identified significant differences between human and animal UPECs. This may have its roots in the fact that it is difficult to determine if an animal has symptoms. Future studies will focus on some of the observations., Competing Interests: Competing Interest The authors declare they have no competing interest.

Polysplenia/heterotaxy syndrome is a rare congenital disorder associated with a wide spectrum of anomalies in various organ systems. Although anomalies of the cardiovascular system are common in this syndrome, the authors report a rare case of polysplenia syndrome associated with aortic pseudocoarctation, which to our knowledge has never been reported., (Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

A 58-year-old man reported sudden-onset binocular double vision that appeared 3 days earlier. He denied history of headache/cervical pain or trauma. He had a medical history of well-controlled diabetes, hypertension and dyslipidaemia. Neurological examination revealed a left-sided ptosis and binocular horizontal diplopia in dextroversion without apparent extraocular-muscle paresis or pupillary involvement. Other cranial nerves were spared as well as motor, sensory and coordination systems. There were no signs of ocular erythema, proptosis or palpable orbital mass. Brain MR angiography revealed a crescent-shaped mural hyperintensity in left internal carotid artery (ICA) at skull base, extending to intrapetrous segment, with reduced calibre and flow, suggesting a left ICA dissection. The patient was started on antiaggregation therapy. A year later he was asymptomatic and CT angiography confirmed ICA recanalisation., (2014 BMJ Publishing Group Ltd.)

Introduction and Objective: This is the first study in Angola with the aim of characterizing ventricular septal defect (VSD) among children and adolescents., Methods: A cross-sectional study based on echocardiographic records of the largest pediatric cardiology center in Angola included all children and adolescents (0 to 18 years old) with VSD between April 2010 and March 2011. The diagnosis was made by transthoracic and Doppler echocardiography with a Medison SA 8000 system. The sample was divided into two groups: Group 1, isolated VSD; and Group 2, VSD associated with other congenital heart defects (CHDs). Age, gender, type of VSD, associated CHDs and genetic syndromes were assessed., Results: A total of 490 CHDs were diagnosed, of which 283 were VSDs. In Group 1 (140, 49%) the mean age was 29±36 months. The most frequent age (mode) at diagnosis was 24 months. There was no predominance of gender (ratio 1:1). The majority (127, 91%) had perimembranous VSD. In Group 2 (143, 51%) 113 patients (79%) had one, 27 patients (19%) had two and three patients (2%) had three other CHDs. Trisomy 21 was the most common genetic syndrome (23, 96%)., Conclusions: The study shows that VSD is the most common CHD in childhood, the diagnosis is made late and almost half of VSDs are associated with other CHDs., (Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Unlabelled: In 2002, Spring et al reported a family with an autosomal dominant form of hereditary sensory neuropathy; patients also presented adult onset of gastroesophageal reflux and cough. Since then, no further families have been described., Objective: To study a new Portuguese family with these characteristics., Method: To describe the clinical and neurophysiologic characteristics of one family with features of sensory neuropathy associated with cough and gastroesophageal erflux., Results: Three of five siblings presented a similar history of paroxysmal cough (5th decade). About a decade later they experienced numbness and paraesthesia in the feet and in all cases there was evidence of an axonal sensory neuropathy. A history of gastroesophageal reflux of variable severity and age of onset was also present., Discussion: Molecular genetic studies have demonstrated genetic heterogeneity between the hereditary sensory neuropathy type 1 subtypes. The identification of these families is of major importance because further work is required to identify the underlying genetic defect.

Prosthetic valve thrombosis (PVT) is a rare but serious complication of valve replacement, most often encountered with mechanical prostheses. The different therapeutic modalities for PVT (fibrinolysis with heparin treatment or surgery) will largely be influenced by the presence of valvular obstruction, the valve location (left or right sided), the patient's clinical status, the existence of and expertise in therapeutic modalities at the institution, and the patient's decision. This report describes a patient with thrombosis of a prosthetic mitral valve, which was successfully treated with recombinant streptokinase in a hospital without cardiac surgery. In this context, the authors present the real-time transoesophageal echocardiographic appearance of this complication, and give a brief review of the literature.

Sinus of Valsalva aneurysms (SVA) are considered rare congenital anomalies. Apart from a few reported cases of SVA in African patients, there is little information on the clinical presentation of this entity in Africans. We describe the clinical and echocardiographic features of four consecutive African patients in whom the diagnosis of SVA was established over the last six years at the echocardiography laboratory of our institution. In this report we compare the clinical and echocardiographic features in our patients with those of others published in the literature.

Submitral ventricular aneurysm is a thoroughly studied pathology but is not well known due to its rarity. Clinically, it is manifested by symptoms and signs of heart failure, mitral regurgitation and/or ventricular arrhythmias, and may be associated with thromboembolic phenomena and myocardial ischemia due to compression of the coronary arteries by the aneurysm. A rare complication of this type of aneurysm is rupture into the left atrium. Transthoracic echocardiography plays an important role in the definitive diagnosis of this pathology, although the role of transesophageal echocardiography in the evaluation of these patients is less known. We report a case of a submitral ventricular aneurysm complicated by rupture into the left atrium, which was diagnosed by transesophageal echocardiography.

Unlabelled: Chronic heart failure (CHF) is a serious public health problem all over the world. CHF has a high prevalence, affecting mainly the elderly, and causes severe disability and social and economic costs., Aim: To estimate the prevalence of chronic heart failure in the Autonomous Region of Madeira in 2001., Methods: This was a community-based epidemiological survey involving subjects attending primary care centers selected by a combined two-stage sampling and stratifying procedure. General practitioners (GPs) randomly selected in proportion to the population of each municipality evaluated subjects aged over 25 years attending primary care centers, recruited consecutively and stratified by age. CHF cases were identified according to the Guidelines of the European Society of Cardiology for CHF diagnosis., Results: Six hundred and eighty-six eligible subjects were evaluated by 30 GPs; 60 patients with CHF were identified. The overall prevalence and 95 % CI of CHF in Madeira was 4.69 % (2.91 % to 6.46 %), 3.53 % in males (0.81 % to 6.26 %), and 5.58 % in females (3.37 % to 7.79 %). CHF prevalence increases with age: 1.24 % (0.00 % to 2.96 %) in the 25 to 49-year-old group, 6.17% (1.31 to 11.03 %) in those aged 50 to 59 years, 7.62 % (0.75 to 14.49 %) in those aged 60 to 69 years, 13.32 % (7.99 % to 14.49 %) in the 70 to 79-year-old group, and 14.34 % in the group over 80 years old (7.59 % to 21.09 %). The prevalence of CHF due to systolic dysfunction was 0.76 % and 2.74 % with normal systolic function., Conclusions: The overall prevalence of CHF in Madeira was similar to that of mainland Portugal, and slightly higher than that of other European studies. Overall CHF prevalence increases sharply with age. The prevalence of CHF with preserved systolic ventricular function was similar to that reported by other recent European studies. The prevalence of CHF due to systolic dysfunction was much lower. The differences found may correspond to differences in methodology rather than actual differences in the population. CHF with left ventricular systolic dysfunction seems to be rare in primary care in Madeira. This may be related to the different public health organization in Madeira, and deserves further evaluation.

Background: Chronic heart failure (CHF) is common and is frequently managed by primary care physicians (PCPs). Despite the European Society of Cardiology (ESC) Guidelines, standard treatments for CHF are frequently underutilised, particularly in primary care., Aim: To evaluate current drug therapy for CHF in adults with HF diagnosed according to ESC guidelines in the context of the EPICA study. Aetiological features and therapy relevant comorbidities were also analysed., Methods: EPICA was a community-based epidemiological study conducted in mainland Portugal. The study involved 365 primary care physicians, who evaluated 6300 primary care attendees aged over 25 years. CHF was diagnosed by clinical and echocardiography criteria according to ESC guidelines., Results: Total of 551 cases of CHF were identified, with a mean age of 65+/-9 years. The estimated overall prevalence of CHF in the Portuguese population was 4.4%; 1.3% with and 1.7% without left ventricular systolic dysfunction (LVSD). There are 6,280,792 people aged >25 years in Portugal, which extrapolates to 261,400 cases of heart failure. About 80% of patients had a history of hypertension, 39% had a history of coronary artery disease and 15% had atrial fibrillation. Only 58% of patients were on angiotensin-converting enzyme (ACE) inhibitors and 7% on beta-blockers. The type of ventricular dysfunction, age and presence of renal failure had little effect on prescription rates. Diuretics were prescribed in 78%. Thiazides were used more frequently in those with preserved systolic function and frusemide in those with left ventricular systolic dysfunction. Digoxin was prescribed more often to patients with than without left ventricular systolic dysfunction (34% vs. 17%; p=0.02). Long-acting nitrates were prescribed to 20% and amiodarone to 8% of patients., Conclusion: The EPICA study, as in other studies in primary care in Europe, particularly the IMPROVEMENT study, suggests that greater efforts are required to improve training of primary care teams in the management of CHF.

Background: There is a common assumption that a normal ECG or a normal heart size on chest X-ray virtually rules out a diagnosis of heart failure., Aims: To assess the value of the electrocardiogram and chest X-ray in identifying patients with chronic heart failure in the community., Methods and Results: This study was a secondary analysis of data prospectively collected at the time of patient's enrollment in the EPICA study, an epidemiological study of the prevalence of heart failure in Portugal. A total of 6300 subjects were clinically evaluated. Patients who presented with symptoms or signs of heart failure, and/or were receiving diuretics for chronic heart failure (CHF) had a chest X-ray, ECG, and echocardiogram. The diagnosis of heart failure was confirmed in 551 cases. Patients with right atrial enlargement, atrial flutter, atrial fibrillation, 2nd degree-Mobitz I atrioventricular block, 1st degree atrioventricular block, left bundle branch block, lung interstitial oedema, and bilateral pleural effusion were more likely to be diagnosed with heart failure. For the diagnosis of heart failure, in the Portuguese population aged over 25 years, an abnormal electrocardiogram had an estimated sensitivity of 81%, and negative predictive value of 75%; an abnormal chest X-ray had an estimated sensitivity of 57%, and negative predictive value of 83%. Twenty five percent of patients with CHF had a normal ECG or chest X-ray., Conclusion: Our results show that electrocardiographic and roentgenographic features are not sufficient to allow heart failure to be reliably predicted in the community and support the recommendation that all patients with suspected heart failure should undergo echocardiography.

Aim: To validate and estimate the performance statistics and concordance of seven clinical questionnaires for the diagnosis of chronic heart failure (HF)., Methods: Community-based epidemiological survey of patients aged >25 years attending a random sample of primary health care centers in Portugal. Heart failure was identified according to the Guidelines of the European Society of Cardiology (ESC)., Results: A total of 5434 subjects evaluated by 365 investigators were eligible for analysis, 551 of which had criteria for heart failure. Overall, the questionnaires had high specificity, usually above 90%, but low sensitivity, providing an increase in the likelihood of heart failure from 4.3% pre-test to 25-35% post-test in most cases. The Göteborg questionnaire was the most balanced regarding sensitivity (84%) and specificity (81%) but this may reflect its use of prescription of digoxin or diuretics as diagnostic criteria for diagnosis. The Walma, Framingham and NHANES-I questionnaires performed similarly (Sensitivity: 63%, Specificity: 93%), while the Boston and the Gheorghiade questionnaires had a somewhat lower sensitivity (55%). Concordance was good between the Boston, Framingham, Gheorghiade, NHANES-I and Walma questionnaires., Conclusions: This study evaluated seven clinical questionnaires for the diagnosis of heart failure in the community. Their low sensitivity impairs their usefulness as diagnostic instruments, but their high specificity makes them useful for the identification of patients with fluid retention and/or exercise intolerance from non-cardiac causes.