The Brazilian Policy of Comprehensive Care for People with Rare Diseases (BPCCPRD) was established by the Ministry of Health to reduce morbidity and mortality and improve the quality of life of people with rare diseases (RD). Several laboratory tests, most using molecular genetic technologies, have been incorporated by the Brazilian Public Health System, and 18 specialised centres have so far been established at university hospitals (UH) in the capitals of the Southern, Southeastern and Northeastern regions. However, whether the available human and technological resources in these services are appropriate and sufficient to achieve the goals of care established by the BPCCPRD is unknown. Despite great advances in diagnosis, especially due to new technologies and the recent structuring of clinical assessment of RD in Brazil, epidemiological data are lacking and when available, restricted to specific disorders. This position paper summarises the performance of a nationally representative survey on epidemiology, clinical status, and diagnostic and therapeutic resources employed for individuals with genetic and non-genetic RD in Brazil. The Brazilian Rare Disease Network (BRDN) is under development, comprising 40 institutions, including 18 UH, 17 Rare Diseases Reference Services and five Newborn Screening Reference Services. A retrospective study will be initially conducted, followed by a prospective study. The data collection instrument will use a standard protocol with sociodemographic data and clinical and diagnostic aspects according to international ontology. This great collaborative network is the first initiative of a large epidemiological data collection of RD in Latin America, and the results will increase the knowledge of RD in Brazil and help health managers to improve national public policy on RD in Brazil.
Background Pericardial defects are rare anatomical variations that can present as an isolated variation or be associated with other conditions. They are usually asymptomatic and misdiagnosed conditions, and given their rarity, partial pericardial defects can have devastating outcomes. The sudden death of an apparently healthy newborn certainly raises concerns, and a medico-legal investigation is crucial in establishing the cause of death. This case report highlights the importance of awareness on the part of obstetric professionals of the lethal outcomes of pericardial partial congenital defects. This case also demonstrates the difficulty of establishing a correct diagnosis. Case presentation The autopsy of a 15-h-old neonate revealed a partial pericardial defect ending in a biventricular strangulation by the defective pericardium. Other findings, such as the patency of the arterial ductus, a subarachnoid hemorrhage, and aspiration of amniotic fluid, were also reported. Conclusions Although imaging techniques have evolved, fetal detection of cardiac abnormalities can be tricky, especially when occurring as an isolated variation.
Background Rheumatoid arthritis (RA) generates an inflammatory profile that predisposes to total and visceral fatty accumulation and reduced fat free mass (FFM). This metabolic disorder contributes to poor functionality, increased cardiovascular risk and higher mortality. This study aimed to address a systematic review with meta-analysis to determine the effect of biological and targeted synthetic disease-modifying antirheumatic drugs (bDMARDs and tsDMARDs) on body composition (BC) of patients with RA. Methods The search was conducted at the electronic databases PubMed, Cochrane Library, Embase, Lilacs and grey literature. This investigation was carried until July 2021. Outcomes of interest were total weight, body mass index (BMI), fat mass (FM) and FFM. A meta-analysis comparing these outcomes in RA patients under bDMARD treatment versus controls was performed. Results Out of 137 studies reviewed, 18 were selected: fifteen prospective cohorts, two retrospective cohorts, and one cross-sectional study. The studies comprised 1221 patients, 778 on bDMARD treatment and 443 controls, which included RA patients under conventional synthetic DMARD (csDMARD). No study addressing BC analysis in patients using tsDMARD was found. The mean age and duration of the disease was 56.7 years and 6.77 years, respectively. Ten studies demonstrated a significant increase of total weight in 88.2% of patients and 42.3% for BMI. In studies that analyzed BC by double X-ray absorptiometry (DXA), the increase in total weight and BMI correlated positively to the increase in FFM. The meta-analysis carried out in five studies showed no significant difference of the mean difference for total weight 0.12 kg (95% CI − 5.58, 5.82), BMI 0.08 kg/m ² (95% CI − 1.76, 1.92), FM − 0.08 kg (95% IC − 5.31, 5.14), and FFM − 2.08 kg (95% CI − 7.37, 3.21). Conclusion This systematic review suggests a possible impact of bDMARDs on BC of RA patients, even though, the meta-analysis carried out in a small part of these studies was not able to confirm significant variation in BC components. Trial registration : PROSPERO code: CRD42020206949.
Background We examined the influence of superoxide dismutase 3 (SOD3) Arg213Gly and Peroxisome Proliferator-Activated α-Receptor (PPARα) 7G/C polymorphisms to a single dose of purple grape juice supplementation on time-to-exhaustion running test, redox balance and muscle damage in recreational runners. Methods Forty-seven male recreational runners performed a running test until exhaustion after supplementation with grape juice or a control drink. Serum total antioxidant capacity (TAC), malondialdehyde (MDA), plasma nitrite (NO), creatine kinase (CK) and lactate dehydrogenase (LDH) were measured pre and post exercise. Also, polymorphisms were analyzed in DNA extracted from the oral mucosa. Results Grape juice improved the time-to-exhaustion. When analyzed by genotype, the recreational runners with GG+CG genotypes of the SOD3 gene had greater time-to-exhaustion than the CC genotype, but was no different for the PAPRα gene. A slight difference was noted in TAC, since the CC genotype of the SOD3 gene showed higher TAC values in the post-exercise compared to the baseline and with pre-exercise, but these values did not increase compared to the CG+GG group, respectively. The SOD3 and PPARα genes were similar at all times for the other biochemical variables. Conclusion The ergogenic effect of grape juice was genotype-dependent for SOD3 Arg213Gly. However, biochemical redox balance markers did not explain this difference.
The emergence of digital pathology environments and the application of computer vision to the analysis of histological sections has given rise to a new area of Anatomical Pathology, termed Computational Pathology. Advances in Computational Pathology may substantially change the routine of Anatomical Pathology laboratories and the work profile of the pathologist.
Despitethe increasing number of publications on residual raw materials as supplementary cementitious materials (SCM), the milling beneficiation process and its parameters have been underexplored and presented. In this context, this study aims to evaluate the milling parameters for marble, clay tile, clay brick, and phosphogypsum waste processing for recycling as SCM. The raw materials were benefitted by grinding, sieving, and milling in a planetary ball mill, varying the time and rotation speed parameters. The waste was characterised by helium gas pycnometry, DSC, BET specific surface area, XRF, TGA, and XRD/Rietveld. Waste materials in which the mineral composition of phases was formed at higher temperatures were associated with higher demands for specific milling energy and lower grindability indexes. Marble waste (MW) has a mineral composition similar to commercial limestone and phosphogypsum (PG) can be an alternative to natural gypsum in cementitious materials. Clay brick waste (CBW) and clay tile waste (CTW) have the potential to be used as SCM to replace calcined natural clays, although CTW requires higher energy during milling processes.
No research demonstrates the effect of combined waste raw materials as an alternative to natural sources in Limestone Calcined Clay Cement (LC3). In this context, this study aimed to evaluate the influence of the composition of ternary cements (TCs) containing industrial waste on their rheological and hydration properties. As raw materials, Portland cement and clay brick (CBW), clay tile (CTW), marble (MW), and phosphogypsum (PG) wastes were used. The rheological behaviour of the pastes was analysed by the mini-slump evolution over time and rotational rheometry. Hydration was evaluated by isothermal calorimetry and XRD/Rietveld. An increase in the specific surface area enhances the yield stress and plastic viscosity of the paste. CBW and CTW have pozzolanic reactivity, presenting an increase in the content of non-crystalline phases, including calcium silicate hydrate (C-S-H). The TCs reached at least 70% of the compressive strength of theHigh Early Strength Portland cement paste.The results suggest that CBW, CTW, MW and PG can be used as an alternative to reduce the clinker factor and decrease the environmental and economic impacts associated with extracting natural raw materials for cement production.
Introduction It is important to clarify the effect of ventilator hyperinflation(VHI) on pulmonary function and secretion clearance in adults receiving mechanical ventilation(MV). There is no published meta-analysis on the effects VHI on pulmonary function and secretion clearance in adults receiving MV. Objective Analyze the published randomized clinical trials(RCTs) that investigated the effects of VHI on pulmonary function and secretion clearance in adults receiving MV, comparing VHI with isolated aspiration, VHI with manual hyperinflation(MHI), VHI +vibrocompression(VB) versus VB and VHI+VB versus isolated aspiration. Methods The following databases PubMed, LILACS, EMBASE, SciELO, PEDro database and Cochrane Central Register of Controlled Trials (CENTRAL) were consulted up to December 2021. Secretion clearance, static and dynamic compliance of the respiratory system(Cstat and Cdyn), airway resistance(Raw) and oxygenation outcomes were evaluated. Results Thirteen studies met the study criteria, but only 12 studies were included on meta-analysis. There was no difference between VHI versus isolated aspiration for amount of secretions removed(0.41 SMD; 95% CI: -0.08 to 0.89; n=270), VHI versus MHI(0.51 grams; 95% CI: -0.08 to 1.11; n=256), VHI+VB versus VB(0.31 grams; 95% CI: -0.42 to 1.05; n=130) and VHI+VB versus isolated aspiration(0.54 grams; 95% CI: -0.06 to 1.14; n=132). There was difference for VHI versus isolated aspiration to Cstat (4.77 ml/cm H2O; 95% CI: 2.41 to 7.14; n= 136). Conclusion No evidence was found that VHI was effective in increasing the amount of secretions removed, Cdyn and oxygenation, but VHI seems to show a slight improvement in Cstat when compared to isolated aspiration.
Isolated Na2O⋅2CaO⋅3SiO2 (NC2S3) single crystals grew at temperatures above glass transition in a melt with a slight soda excess. The enrichment of Na in the continuous solid solution Na4+2zCa4–z[Si6O18] (with 0 ≤ z ≤ 1) upon crystallisation causes a depletion of soda of the growing interfacial diffusion zone. Therefore its viscosity will change. Experimental data and idealized model calculations of the viscosity show the same trend and indicate that the viscosity of the interfacial zone would be up to several orders of magnitude higher than the viscosity of the melt. This increased viscosity at the crystal growth front and the experimentally observed decrease of the crystallisation velocity with crystal size suggest a linear increase of the effective activation enthalpy of the diffusional transport through the diffusion zone with crystal size, which allows to quantitatively model crystal growth data of the NC2S3 glass investigated.
A standalone photovoltaic-battery system (SBPS) for remote areas must be reliable, cost-effective, safe, and designed to extend battery life. A typical configuration of SPBS is non-isolated and uses a dc–dc bidirectional converter for charging and discharging the batteries connected to the dc link. This configuration needs a high-gain dc–dc converter, usually inefficient, to integrate the batteries to the dc link or stack batteries in series to obtain high voltage. This paper proposes connecting the batteries, through a bidirectional low ripple current converter (Cuk), to the terminals of the photovoltaic (PV) panel to solve the problems mentioned above. The Cuk bidirectional converter controls the dc-link voltage by charging/discharging the battery using simple cascade control. In addition to managing the power battery, the continuous currents at the input and output of the converter avoid excessive current oscillations at the PV panels and battery terminals, improving the maximum power point tracking (MPPT) performance and the battery lifetime. The proposed SBPS is composed of a combination of an isolated interleaved boost (IIB) converter, a Cuk bidirectional converter, and a 3-Level T-type (3LT2) Neutral-Point Clamped (NPC) inverter to reduce the number of switches and assure power quality. This configuration provides grounding and isolation between PV panels, battery, and the load. The analysis of power converters that compose the SBPS, the dynamic behavior, and the design procedures are introduced and verified by simulations and experimental results obtained from a Hardware-in-the-Loop (HIL) testing platform.
In this work, we consider the piezoelectric beams model with second sound, that is, the model includes thermal effect given by the Cattaneo’s law of heat conduction. Existence and uniqueness of solutions of the system is proved by the semigroup theory. We prove that the system is exponentially stable using the energy method with the multiplier techniques. This result is obtained without depending on any relationship between the coefficients.
Licuri cake (LC) is an interesting alternative nutritional component for high-grain diets for ruminants due to its high protein (21.6% CP), fat (12.0% EE), and fiber (62.8% NDF) contents. We investigated the effect of increasing levels of LC inclusion on the intake, digestibility, feeding behavior, performance, and carcass traits of cull cows fed high-grain diets. Forty Zebu cows with an initial weight of 318 ± 38.1 kg, at 105 months of age, were confined in collective stalls and fed (ad libitum) a diet containing 0, 50, 100, or 150 g/kg LC on a diet dry matter (DM) basis. Licuri cake inclusion induced quadratic responses (P < 0.05) in the intakes of DM (9.57 kg/day at 97.8 g/kg LC), crude protein (CP; 1.02 kg/day at 91.7 g/kg LC), and total digestible nutrients (6.68 kg/day at 75.2 g/kg LC). The digestibility of DM, CP, and non-fibrous carbohydrates decreased linearly (P < 0.05). Neutral detergent fiber (NDF) intake and rumination efficiencies increased linearly (P < 0.05) with the inclusion of LC in the diet. The inclusion of LC did not influence (P > 0.05) on daily weight gain, hot carcass weight, or back fat thickness in the carcass. Therefore, we recommend the inclusion of up to 150 g/kg LC in high-grain diets for feedlot cull cows.
Being plastic in face of environmental changes that negatively affect communities can increase the average fitness within populations and alter the resilience of the ecological system. Understanding this effect is important for making predictions about future landscapes. We used simulation studies to assess the effects of dispersal behaviour plasticity on community resilience, in the context of disturbance (habitat loss and fragmentation) caused by humans. We also evaluated how resilience is affected by the cost of plasticity, trophic level and specialisation, and by the extent and clustering of the disturbance, through an agent-based model with 10 model species and 3 trophic levels. We found that community resilience is best explained by plasticity, plasticity cost, disturbance extent, and disturbance clustering, with increased community resilience when the individuals have plasticity. This effect increases with disturbance extent – the resilience increases as the degree of plasticity increases. Herbivores and generalists were more resilient than carnivores and specialists. We conclude that all explanatory variables used in this study are relevant to resilience in a world suffering rapid changes caused by humans.
The objective of the study was to evaluate the intake, rumen and hematological parameters, milk composition and milk yield of dairy goats fed diets containing Lippia alba hay as phytogenic feed additive. Eight multiparous crossbred Alpine goats, at an average lactation stage of 50 ± 4.5 days after kidding were used, distributed in a double Latin square design with four treatments [Lippia alba hay as phytogenic feed additive included at levels 0, 30, 60, and 90 g/kg of total dry matter (DM)]. Lippia alba inclusion linearly increased (P < 0.05) the daily intake of DM and acid detergent fiber, milk yield (MY), corrected MY3.5%, corrected-solids, and milk contents (g/d) of protein, fat, total solids, and defatted dry extract daily yield, feed efficiency (MY/DMI ratio) and MY/CP (crude protein) intake ratio of the dairy goats. In contrast, the intake of NFC, NDFap and total carbohydrates reduced (P < 0.001) due to Lippia alba hay as phytogenic feed additive in the goats’ diet. The inclusion of Lippia alba as phytogenic feed additive in the diet of the dairy goats did not affect (P > 0.05) the CP, ether extract and crude ash intake, goat milk concentrations (%) of protein, fat, total solids and defatted dry extract, as well as cryoscopic index, pH, density and acidity of milk, red blood cells count, leukocytes count, packed cell volume, total plasmatic proteins, and rumen values of temperature (°C), pH, and methylene blue reduction, flotation/sedimentation time, color, and consistency of goat rumen fluid. However, the inclusion of Lippia alba hay as a microbial additive reduced moderate (++) and increased abundant (+++) protozoa motility and reduced the density of medium size protozoa, and increased large size protozoa count (P < 0.05). The addition of moderate amounts (up to the level of 90 g/kg total diet) of Lippia alba hay as a phytogenic feed additive in the diet of dairy goats is recommended because it has favorable effects on intake, milk yield, and solids content, with no negative effects on rumen and hematological parameters.
Background Racism is a social determinant of health inequities. In Brazil, racial injustices lead to poor outcomes in maternal and child health for Black and Indigenous populations, including greater risks of pregnancy-related complications; decreased access to antenatal, delivery, and postnatal care; and higher childhood mortality rates. In this study, we aimed to estimate inequalities in childhood mortality rates by maternal race and skin colour in a cohort of more than 19 million newborns in Brazil. Methods We did a nationwide population-based, retrospective cohort study using linked data on all births and deaths in Brazil between Jan 1, 2012, and Dec 31, 2018. The data consisted of livebirths followed up to age 5 years, death, or Dec 31, 2018. Data for livebirths were extracted from the National Information System for livebirths, SINASC, and for deaths from the Mortality Information System, SIM. The final sample consisted of complete data for all cases regarding maternal race and skin colour, and no inconsistencies were present between date of birth and death after linkage. We fitted Cox proportional hazard regression models to calculate the crude and adjusted hazard ratios (HRs) and 95% CIs for the association between maternal race and skin colour and all-cause and cause-specific younger than age 5 mortality rates, by age subgroups. We calculated the trend of HRs (and 95% CI) by time of observation (calendar year) to indicate trends in inequalities. Findings From the 20 526 714 livebirths registered in SINASC between Jan 1, 2012, and Dec 31, 2018, 238 436 were linked to death records identified from SIM. After linkage, 1 010 871 records were excluded due to missing data on maternal race or skin colour or inconsistent date of death. 19 515 843 livebirths were classified by mother's race, of which 224 213 died. Compared with children of White mothers, mortality risk for children younger than age 5 years was higher among children of Indigenous (HR 1·98 [95% CI 1·92–2·06]), Black (HR 1·39 [1·36–1·41]), and Brown or Mixed race (HR 1·19 [1·18–1·20]) mothers. The highest hazard ratios were observed during the post-neonatal period (Indigenous, HR 2·78 [95% CI 2·64–2·95], Black, HR 1·54 [1·48–1·59]), and Brown or Mixed race, HR 1·25 [1·23–1·27]) and between the ages of 1 year and 4 years (Indigenous, HR 3·82 [95% CI 3·52–4·15]), Black, HR 1·51 [1·42–1·60], and Brown or Mixed race, HR 1·30 [1·26–1·35]). Children of Indigenous (HR 16·39 [95% CI 12·88–20·85]), Black (HR 2·34 [1·78–3·06]), and Brown or Mixed race mothers (HR 2·05 [1·71–2·45]) had a higher risk of death from malnutrition than did children of White mothers. Similar patterns were observed for death from diarrhoea (Indigenous, HR 14·28 [95% CI 12·25–16·65]; Black, HR 1·72 [1·44–2·05]; and Brown or Mixed race mothers, HR 1·78 [1·61–1·98]) and influenza and pneumonia (Indigenous, HR 6·49 [95% CI 5·78–7·27]; Black, HR 1·78 [1·62–1·96]; and Brown or Mixed race mothers, HR 1·60 [1·51–1·69]). Interpretation Substantial ethnoracial inequalities were observed in child mortality in Brazil, especially among the Indigenous and Black populations. These findings demonstrate the importance of regular racial inequality assessments and monitoring. We suggest implementing policies to promote ethnoracial equity to reduce the impact of racism on child health. Funding MCTI/CNPq/MS/SCTIE/Decit/Bill & Melinda Gates Foundation's Grandes Desafios Brasil, Desenvolvimento Saudável para Todas as Crianças, and Wellcome Trust core support grant awarded to CIDACS-Center for Data and Knowledge Integration for Health.
The presence of produced water is common in oil production. In certain production stages, the volume of produced water exceeds that of oil. Pretreatment is necessary before the disposal or reinjection of produced water into wells because it is harmful to the environment owing to the high oil and grease contents (OGC), salinity, and other factors. This study proposes a produced water treatment using microemulsions (ME) with vegetable oil-based surfactants (S), with the aim of reducing two main factors, OGC and salinity. The decision to use a vegetable oil-based surfactant as an alternative to a petroleum-based surfactant was made to reduce the negative effects of the overall process on the environment. Coconut soap, the surfactant used in the formulated ME, was selected after testing a variety of vegetable oil-based surfactants. The other components used in the ME formulation were 2-butanol as a co-surfactant (C), pine oil as the oil phase, and saline water at 2 % NaCl as the water phase. The ME point used in the water treatment was defined by D-optimal mixture planning, composed of 85 % C/S, 2.5 % oil phase, and 12.5 % water phase, with a C/S ratio of 10. In addition, we used a complete factorial experimental design with combinations of three variables (time, temperature, and microemulsion percentage), and repetition of the central point to select the greatest reduction in OGC and salinity. According to the results, OGC reduction values ranged from 89.15 % to 95.43 %, with the real OGC values ranging from 16.98 mg·L⁻¹ to 7.15 mg·L⁻¹. These values are within the limits established by environmental agencies, CONAMA, US EPA, and the OSPAR Commission, for the disposal of produced water in the sea. In addition, salinity reduction varied from 38.02 % to 46.8 %, with real salinity decreasing from 36,223.53 mg·L⁻¹ to 31,217.65 mg·L⁻¹. Therefore, it can be said that using a vegetable oil-based and biodegradable surfactant in the microemulsion formulation for the treatment of produced water is as effective in the reduction of OGC and salinity as petroleum-based surfactants, which are more harmful to the environment.
Resumo O objetivo é analisar a organização e oferta de atenção especializada (AE) e transporte sanitário nas Policlínicas Regionais da Bahia, via Consórcios Interfederativos de Saúde. Foi realizado estudo de caso em uma Policlínica, com abordagem qualitativa, a partir de entrevistas com atores municipais e estaduais. Buscou-se identificar elementos que caracterizam um modelo de AE integrado às Redes de Atenção à Saúde. Entre os avanços identifica-se ampliação da oferta de AE; garantia de escopo, qualidade e fixação de profissionais; provisão de transporte sanitário; monitoramento da prestação da AE; e avanços na regulação por sistema informatizado. Representam desafios: adequação do planejamento da AE à realidade locorregional; incentivo à coordenação do cuidado pela Atenção Primária à Saúde (APS) e à aproximação entre profissionais da AE e APS; institucionalização da contrarreferência, relação com instâncias de participação social; e incentivo às funções de matriciamento, Educação Permanente, integração ensino-serviço e pesquisa. Considera-se o arranjo das Policlínicas Regionais, mais afeito à integração às Redes de Atenção à Saúde, em que pese os desafios intrínsecos à AE e ao necessário fortalecimento da APS para que possa assumir a condução do sistema.
The aim of this study was to analyze the organization and provision of specialized care (SC) and health transport in regional polyclinics in the state of Bahia, Brazil. We conducted a qualitative exploratory single case study of a polyclinic based on semi-structured interviews with key informants in municipal and state health services. We sought to identify elements that characterize network-based models of SC. A number of advances were identified, including: an increase in the provision of SC and the scope and quality of services; effective retention of health professionals; provision of health transport; monitoring of SC; and improvements in the regulation of access to care through the use of computerized systems. The following challenges were observed: tailoring SC planning to regional health needs; the promotion of care coordination by Primary Health Care (PHC); the development of strategies to improve communication between SC and PHC professionals; institutionalization of counter-referral; engagement between the polyclinic and spaces for citizen participation; and fostering matrix support, permanent education, teaching-service integration, and research. Despite the intrinsic challenges of SC and the need to strengthen the central role of PHC, the regional polyclinic arrangement is better suited to the integration of the health care networks.
The study aimed to characterize women that attended prenatal care in Brazil according to ethnicity/skin color and sociodemographic variables and to verify the association between the indicators of the prenatal care process and the women’s ethnicity/skin color. This was a population based, cross-sectional study carried out with data from the National Health Survey of 2013. A bivariate analysis was performed using the multilevel logistic regression model, estimating the odds ratio and the respective 95% confidence intervals to test the association between the indicators of the adequate prenatal care process and the women’s ethnicity/skin color. The findings showed that black women have a lower chance of starting prenatal care before 12 weeks of gestation, having 6 or more consultations, performing the HIV test, performing the VDRL exam or receiving advice related to care during gestation and childbirth. Inequalities were identified in the healthcare of Brazilian women during prenatal care, related to ethnicity/skin color and other sociodemographic characteristics. It was concluded that being of black ethnicity and living in a socially disadvantaged area entails disadvantages for women regarding access to a prenatal care considered to be adequate according to the criteria established by the Brazilian Ministry of Health.
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Rua Augusto Viana, s/n - Palácio da Reitoria, Canela, 40110-909, Salvador, Bahia, Brazil
Head of institution
João Carlos Salles Pires da Silva
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