Influence of the timing of biological treatment initiation on Juvenile Idiopathic Arthritis long-term outcomes.

BACKGROUND: Juvenile idiopathic arthritis (JIA) treatment is aimed at inducing remission to prevent joint destruction and disability. However, it is unclear what is the long-term impact on health-related outcomes of the timing of biological disease-modifying antirheumatic drug (bDMARD) initiation in JIA. Our aim was to evaluate the long-term impact of the time between JIA onset and the initiation of a bDMARD in achieving clinical remission, on physical disability and health-related quality of life (HRQoL). METHODS: Adult JIA patients registered in the Rheumatic Diseases Portuguese Register (Reuma.pt) and ever treated with bDMARD were included. Data regarding socio-demographic, JIA-related characteristics, disease activity, physical disability (HAQ-DI), HRQoL (SF-36), and treatments were collected at the last visit. Patients were divided into 3 groups (≤ 2 years, 2-5 years, or > 5 years), according to the time from disease onset to bDMARD initiation. Regression models were obtained considering remission on/off medication, HAQ-DI, SF-36, and joint surgeries as outcomes and time from disease onset to bDMARD start as an independent variable. RESULTS: Three hundred sixty-one adult JIA patients were evaluated, with a median disease duration of 20.3 years (IQR 12.1; 30.2). 40.4% had active disease, 35.1% were in remission on medication, and 24.4% were in drug-free remission; 71% reported some degree of physical disability. Starting a bDMARD > 5 years after disease onset decreased the chance of achieving remission off medication (OR 0.24; 95% CI 0.06, 0.92; p = 0.038). Patients who started a bDMARD after 5 years of disease onset had a higher HAQ and worse scores in the physical component, vitality, and social function domains of SF-36, and more joint surgeries when compared to an earlier start. CONCLUSION: Later initiation of bDMARDs in JIA is associated with a greater physical disability, worse HRQoL, and lower chance of drug-free remission in adulthood.

Tratamento transcateter sequencial de estenose aórtica e disfunção de bioprótese mitral - os limites da cardiologia intervencionista / Sequential transcatheter treatment of aortic stenosis and mitral bioprosthesis dysfunction - the limits of interventional cardiology

APRESENTAÇÃO CLÍNICA JOM, 73 anos, sexo feminino HAS, FA permanente + BRE Submetida em 11/21 a troca valvar mitral (Sorin 23 mm) por estenose mitral reumática. No intraoperatório, foi observada estenose aórtica grave. Devido a CEC prolongada não foi abordada a estenose aórtica Evolui com CF NYHA III apesar de tratamento clínico otimizado ECO um ano após: estenose aórtica grave e dupla disfunção da bioprótese mitral, com estenose grave, regurgitação moderada a grave e PSAP de 45 mmHg Coronariografia sem lesões obstrutivas Euroscore II de 9,48% (STS não contempla procedimento em 2 válvulas) Optado por pela terapia transcateter sequencial devido a alto risco cirúrgico. Tomada de decisão Decidido por realizar o tratamento em suas etapas: TAVI e depois VIV mitral Aliviar primeiro a via de saída do VE, uma vez que o tratamento da doença mitral poderia sobrecarregar com volume um VE trabalhando em regime de pressão elevada 1 semana de intervalo entre as terapias Procedimento - TAVI Punção bi-femoral guiada por ultrassonografia. Leak paravalvar mínimo e gradientes médio de 3 mmHg e máximo de 5 mmHg. CONCLUSÃO É possível realizar o tratamento de valvopatias combinadas transcateter em pacientes de alto risco cirúrgico O planejamento cuidadoso com TC é essencial Esse caso ilustra os limites da Cardiologia Intervencionista sendo superados cada vez mais.

Duplo acesso femoral unilateral: solução simples para tortuosidade ílio-femoral extrema no implante transcateter de válvula aórtica / Double unilateral femoral access: simple solution for extreme ilio-femoral tortuosity in transcatheter aortic valve implantation

APRESENTAÇÃO CLÍNICA UB, 83 anos, sexo feminino HAS, DLP, hipotireoidismo Dispnéia e angina aos pequenos esforços (CF III de NYHA) ECO: estenose aórtica severa, função sistólica normal, área valvar de 0,51 cm2, gradientes médio e máximo de 53 e 85 mmHg, com discreta regurgitação aórtica, pressão sistólica da artéria pulmonar de 45 mmHg Coronariografia sem lesões obstrutivas STS 4,94% // Euroscore II 5,67% Devido a idade, decidido por TAVI em Heart Team. E o acesso? Duplo acesso femoral ipsilateral à direita (dupla punção): Punções guiadas por US Começar pela punção superior com bainha 9F Aplicação de 2 proglides Punção inferior com bainha 5F para pigtail e bailout vascular Guia Lunderquist para suporte Caso necessário, buddy wire com Lunderquist no pigtail. CONCLUSÃO A tortuosidade ílio-femoral intensa está associada ao aumento de risco de complicações vasculares, logo deve-se estar preparado para tratamento de resgate endovascular A tortuosidade também dificulta a navegabilidade da válvula, eventualmente sendo necessário o uso de buddy-wire A dupla punção femoral unilateral é uma solução simples para essa situação anatômica complexa, permitindo tanto o bailout vascular como o buddy wire. Outra opção seria a punção da bainha calibrosa (ex: iSleeve)

Precauções básicas do controlo da infeção: Contributos para a melhoria das práticas em contexto pré-hospitalar / Standard precautions: Contributions to improve pre-hospital practices

Os cuidados de saúde em ambiente pré-hospitalar devem cumprir as recomendações baseadas em evidência, impondo-se a necessidade de implementar uma prática segura que minimize a transmissão de agentes infeciosos para os doentes ou para o próprio prestador de cuidados. As precauções básicas do controlo da infeção são medidas fundamentais para prevenir a transmissão de agentes potencialmente infeciosos, a aplicar em todas as situações de cuidados de saúde, nomeadamente no pré-hospitalar. O estudo tem como finalidade analisar os aspetos organizacionais do contexto pré-hospitalar e avaliar os conhecimentos dos profissionais de saúde (bombeiros, técnicos de emergência do pré-hospitalar, médicos e enfermeiros) no âmbito das precauções básicas do controlo da infeção e das medidas de isolamento. Trata-se de um estudo do tipo observacional, descritivo, correlacional e transversal, com uma amostra de 317 profissionais, de ambos os sexos e média de idade de 37.9 (DP=7.04), que exercem funções em Portugal Continental. O instrumento de colheita de dados foi construído especificamente para este estudo, com base na revisão da literatura e inclui três domínios: caraterização sociodemográfica e profissional, organização do serviço no âmbito do controlo de infeção, conhecimentos relacionados com as precauções básicas do controlo da infeção e as medidas de isolamento. Os resultados revelaram, a nível organizacional, fatores favoráveis ao controlo de infeção, nomeadamente, estratégias de sensibilização para a higiene das mãos e etiqueta respiratória, assim como disponibilidade de equipamento de proteção individual e de solução antissética de base alcoólica, embora com margem para melhoria na organização dos profissionais dedicados ao controlo de infeção. Em termos formativos a maioria dos participantes teve formação em controlo de infeção nos últimos cinco anos, no entanto, essa formação não se espelha nos conhecimentos demonstrados. Os resultados revelaram que os participantes detêm um conhecimento globalmente suficiente em relação às precauções básicas, com um valor médio de 10,3 (DP=1.88), para um total de 15 questões, traduzindo 68.86% de respostas corretas, contudo, o conhecimento foi insuficiente em relação às medidas de isolamento, com um valor médio de 3.1 (DP=1.96), para um total de oito questões, e uma percentagem de apenas 38,75% de respostas corretas. Foi ainda estudada a associação entre variáveis com resultados estatisticamente pouco significativos. As necessidades formativas identificadas pelos participantes vão ao encontro dos défices de conhecimentos revelados. Os resultados obtidos apontam áreas formativas emergentes no âmbito dos conhecimentos referentes às precauções básicas e às medidas de isolamento, e traduzem a necessidade de implementar um plano de formação abrangente, incluindo todos os grupos profissionais com o intuito de melhorar os conhecimentos e facilitar a sua translação para a prática clínica. Futuramente será oportuno a realização de mais estudos que incluam também auditorias às práticas, para facilitar uma compreensão mais completa das necessidades formativas.

Health care in a pre-hospital setting must comply with the evidence-based recommendations, imposing the need to implement a safe practice that minimizes the transmission of infectious agents to patients or to the care provider. Standard Precautions are crucial measures to prevent the transmission of potentially infectious agents, and to apply in all health care situations, namely in pre-hospital care. The study aims to analyse the organizational aspects of the pre-hospital setting and to assess the knowledge of health professionals (firefighters, pre-hospital emergency technicians, doctors and nurses) within the scope of basic precautions and isolation precautions. It is an observational, descriptive, correlational and cross-sectional study, with a sample of 317 professionals, of both genders, and an average age of 37.9 years (SD=7.04), who work in mainland Portugal. The instrument used for data collection was developed specifically for this study, based on the literature review, and includes three domains: sociodemographic and professional characterization, service organization in the context of infection control, knowledge related to standard precautions and isolation precautions. Results showed, at an organizational level, factors favourable to infection control, namely awareness strategies for hand hygiene and respiratory etiquette, as well as availability of personal protective equipment and alcohol-based antiseptic solution, although with room for improvement in the organization of professionals dedicated to infection control. Regarding training, most participants received training in infection control in the last five years; however, this training is not reflected in the knowledge professionals showed. Results evidences that participants have a globally sufficient knowledge about standard precautions, with an average score of 10.3 (SD =1.88), for a total of 15 questions, translating 68.86% of correct answers, however, knowledge was insufficient in what concerns the isolation precautions, with an average score of 3.1 (SD =1.96), in a total of eight questions, a percentage of only 38.75% of correct answers. It was also studied the association between variables, with statistically non-significant results. The training needs identified by the participants fall in line with the revealed knowledge deficits. The obtained results point out emerging formative areas within the scope of knowledge regarding standard precautions and isolation precautions, and translate the need to implement a comprehensive training plan, including all professional groups, aiming to improve knowledge and ease its translation for clinical practice. In the future, it will be important to carry out more studies that also include audits of practices, in order to facilitate a more complete understanding of the training needs

Ionic concentration in periradicular medium after dissolution of endodontic file fragments: an in vitro study.

The aim of this study was to obtain ionic quantification in periradicular medium after diffusion tests of the solution used inside root canals during the electrochemical dissolution of endodontic file fragments and the NiTi-containing dissolution product via an apical foramen. Thirty single-rooted extracted human teeth had root canals prepared and were attached to Eppendorf tubes filled with sterile saline. The samples were divided into 3 groups (n = 10) according to the solution used inside the root canal during the diffusion tests: Group 1: [NaF 12 g/L + NaCl 1 g/L]; Group 2: [NaF 12 g/L + NaCl 1 g/L + NiTi 0.50 g/L]; Group 3: [NaF 6 g/L + NaCl 0.5 g/L + NiTi 0.25 g/L]. The sample in each Eppendorf tube was then analyzed to assay the ionic quantification in periradicular medium. The groups were compared in relation to ionic quantifications (Kruskal-Wallis and Dunn's tests, p ≤ 0.05). Group 2 showed significantly higher F-, Ni and Ti quantities than groups 1 and 3 (p < 0.05). Group 3 showed significantly higher Ti and Ni quantities than group 1, where no measurable quantities of Ti and Ni were observed (p < 0.05). The conclusions were that a 50% dilution of the NiTi-containing dissolution product resulted in significantly lower F-, Ni and Ti quantities compared to the undiluted product. The quantifications observed here suggest that irrigation is recommendable during the electrochemical dissolution process to reduce the resultant ion concentrations in both the root canal and the periradicular medium.

Ionic concentration in periradicular medium after dissolution of endodontic file fragments: an in vitro study

Abstract The aim of this study was to obtain ionic quantification in periradicular medium after diffusion tests of the solution used inside root canals during the electrochemical dissolution of endodontic file fragments and the NiTi-containing dissolution product via an apical foramen. Thirty single-rooted extracted human teeth had root canals prepared and were attached to Eppendorf tubes filled with sterile saline. The samples were divided into 3 groups (n = 10) according to the solution used inside the root canal during the diffusion tests: Group 1: [NaF 12 g/L + NaCl 1 g/L]; Group 2: [NaF 12 g/L + NaCl 1 g/L + NiTi 0.50 g/L]; Group 3: [NaF 6 g/L + NaCl 0.5 g/L + NiTi 0.25 g/L]. The sample in each Eppendorf tube was then analyzed to assay the ionic quantification in periradicular medium. The groups were compared in relation to ionic quantifications (Kruskal-Wallis and Dunn's tests, p ≤ 0.05). Group 2 showed significantly higher F-, Ni and Ti quantities than groups 1 and 3 (p < 0.05). Group 3 showed significantly higher Ti and Ni quantities than group 1, where no measurable quantities of Ti and Ni were observed (p < 0.05). The conclusions were that a 50% dilution of the NiTi-containing dissolution product resulted in significantly lower F-, Ni and Ti quantities compared to the undiluted product. The quantifications observed here suggest that irrigation is recommendable during the electrochemical dissolution process to reduce the resultant ion concentrations in both the root canal and the periradicular medium.

Health-related quality of life and disability in adults with juvenile idiopathic arthritis: comparison with adult-onset rheumatic diseases.

OBJECTIVE: To compare physical disability, mental health, fatigue and health-related quality of life (HRQoL) across juvenile idiopathic arthritis (JIA) categories in adulthood and between JIA and adult-onset rheumatic diseases. METHODS: Cross-sectional analysis nested in a cohort of adult patients with JIA registered in the Rheumatic Diseases Portuguese Register (Reuma.pt). Physical disability (Health Assessment Questionnaire-Disability Index), mental health symptoms (Hospital Anxiety and Depression Scale), fatigue (Functional Assessment of Chronic Illness Therapy-Fatigue Scale (FACIT-F)) and HRQoL (EuroQol-5D (EQ5D) and Short Form (SF-36)) were compared across JIA categories. Patients with polyarticular JIA and enthesis-related arthritis (ERA) JIA were compared respectively to patients with rheumatoid arthritis (RA) and spondyloarthritis (SpA), matched for gender and age, adjusted for disease duration and activity. RESULTS: 585 adult patients with JIA were included. Comparison across JIA categories showed that persistent oligoarthritis and patients with ERA reported a higher score in EQ5D and SF-36 physical component when compared with other JIA categories.Polyarticular JIA reported less disability and fatigue than patients with RA (median Health Assessment Questionnaire of 0.25 vs 0.63; p<0.001 and median FACIT-F score 42 vs 40 ; p=0.041). Polyarticular JIA had also better scores on EQ5D and all domains of SF-36, than patients with RA. Patients with ERA reported less depression and anxiety symptoms (0% vs 14.8%; p=0.003% and 9% vs 21.3%; p=0.002) and less fatigue symptoms (45 vs 41; p=0.01) than patients with SpA. CONCLUSION: Persistent oligoarticular JIA and ERA are the JIA categories in adulthood with better HRQoL. Overall, adult polyarticular and patients with ERA JIA have lower functional impairment and better quality-of-life than patients with RA and SpA.

Corrosion induced fracture of NiTi wires in simulated oral environments.

This work aims to assess the influence of corrosion on fracture of nickel titanium (NiTi) superelastic wires in physiological solutions (9 g/l NaCl) with and without addition of 1 g/l NaF. The electrochemical cell was coupled to a Hounsfield Tensiometer tensile machine commonly used for corrosion investigation of alloys under stress and strain. Corrosion tests were performed on unstrained and strained conditions up to 4% total strain. This strain limit corresponds to 50% of the total elongation achieved into the superelastic stress plateau of the alloy. All wire specimens were analyzed after testing by scanning electron microscopy (SEM). The results showed that localized corrosion occurred for NiTi wires in solution containing fluoride, while no corrosion attack was detected in NaCl 9 g/l solution. There was no significant difference between the corrosion resistance of unstrained and strained wires. However, brittle like fracture occurred in NaCl + NaF solution within the superelastic domain of the material. The most relevant conclusion achieved is that the use of superelastically strained NiTi in oral environments in the presence of fluoride is followed by significant risk of corrosion induced fracture.

Determinants of Discordance Between Criteria for Inactive Disease and Low Disease Activity in Juvenile Idiopathic Arthritis.

OBJECTIVE: To assess concordance among criteria for inactive disease (ID) and low disease activity (LDA) in juvenile idiopathic arthritis (JIA) and to seek factors driving discordance. METHODS: The frequency of fulfillment of existing criteria was evaluated in information on 10,186 patients extracted from 3 cross-sectional data sets. Patients were divided up according to the functional phenotypes of oligoarthritis and polyarthritis. Concordance between criteria was examined using weighted Venn diagrams. The role of each individual component in explaining discordance between criteria was assessed by calculating the absolute number and percentage of instances in which the component was responsible for discrepancy between definitions. RESULTS: Criteria for ID were met by 28.6-41.1% of patients with oligoarthritis and by 24.0-33.4% of patients with polyarthritis. Criteria for LDA were met by 44.8-62.4% of patients with oligoarthritis and by 44.6-50.4% of patients with polyarthritis. There was a 57.9-62.3% overlap between criteria for ID and a 67.9-85% overlap between criteria for LDA. Parent and physician global assessments and acute-phase reactants were responsible for the majority of instances of discordance among criteria for ID (8.7-15.5%, 10.0-12.3%, and 10.8-17.3%, respectively). CONCLUSION: We found fair concordance between criteria for ID and LDA in JIA, with the main drivers of discordance for ID being physician and parent global assessments and acute-phase reactants. This observation highlights the need for further studies aimed to evaluate the impact of subjective physician and parent perception of disease remission and of laboratory measures of inflammatory activity on the definition of ID.

The most important fishery resource in the Amazon, the migratory catfish Brachyplatystoma vaillantii (Siluriformes: Pimelodidae), is composed by an unique and genetically diverse population in the Solimões-Amazonas River System

The migratory catfish Brachyplatystoma vaillantii is one of the most important fishery resources in the Amazon. Intense capture occurs associated to its life cycle. In order to know the genetic status, we sequenced the mitochondrial DNA control region from 150 individuals of B. vaillantii, collected in five fishing landing locations, covering the length of the Solimões-Amazonas River in Brazil. Genetic diversity parameters suggest there is no genetic differentiation between the five localities. Population's expansion indicated by R 2 and Fu's Fs tests was also confirmed by the high number of unique haplotypes found. The Analyses of molecular variance indicated that nearly all variability was contained within locations (99.86%), and estimates of gene flow among B. vaillantii were high (F ST = 0.0014). These results suggest that Brachyplatystoma vaillantii forms a panmitic population along the Solimões-Amazonas River and, has greater genetic variability than other species of the Brachyplatystoma genus available so far. Although the influence of different tributaries on B. vaillantii migration patterns remains uncertain, a single population in the main channel should be consider in future policies for management of this resource. However, since the species' life cycle uses habitats in several countries, its management and conservation depend greatly of internationally joined efforts.(AU)

O bagre migrador, Brachyplatystoma vaillantii, é um dos mais importantes recursos pesqueiros da Amazônia. Intensa captura ocorre associada ao seu ciclo de vida. Para conhecer seu status genético, sequenciamos a região de controle do DNA mitocondrial de 150 indivíduos, coletados em cinco locais de desembarque pesqueiro, abrangendo toda a extensão do rio Solimões-Amazonas no Brasil. Os parâmetros de diversidade genética sugerem que não existe diferenciação genética entre as cinco localidades amostradas. A expansão populacional indicada pelos testes R 2 e Fs de Fu, também foi confirmada pelo elevado número de haplótipos únicos encontrados. A análise de variância molecular indicou que quase toda a variabilidade estava contida nas localidades (99,86%), e as estimativas de fluxo gênico desta espécie eram altas (F ST = 0,0014). Esses resultados sugerem que Brachyplatystoma vaillantii forma uma população panmítica ao longo do rio Solimões-Amazonas com maior variabilidade genética que outras espécies do gênero Brachyplatystoma disponíveis no momento. Embora a influência dos diferentes afluentes na migração de B. vaillantii permaneça incerta, em futuras políticas de gestão deste recurso deve-se considerá-lo como uma única população no canal principal. Entretanto, uma vez que seu ciclo de vida abrange habitats em vários países, seu manejo e conservação dependem muito de esforços internacionais em conjunto.(AU)

Acute toxicity of a deltamethrin based pesticide (DBP) to the Neotropical electric fish Microsternarchus cf. bilineatus (Gymnotiformes)

A deltametrina é um inseticida piretróide amplamente utilizado no controle de pragas na agricultura brasileira. O uso intensivo e desordenado desse pesticida na Amazônia pode carreá-lo aos ecossistemas aquáticos de várias maneiras, mas principalmente por escoamento e lixiviação. O presente estudo foi concebido para determinar a toxicidade aguda (LC50) de um pesticida à base de deltametrina (PBD) e caracterizar seus efeitos sobre dois biomarcadores bioquímicos, a glutationa-S-transferase (GST) e a acetilcolinesterase (AChE), em tecidos do peixe elétrico Microsternarchus cf. bilineatus. Os peixes foram expostos a concentrações de 1, 2, 3, 4 e 5 µg L-1 de PBD por até 96 horas. Para cada tratamento, foi analisada a atividade absoluta das enzimas GST (músculo e fígado) e AChE (músculo e tecido nervoso). A CL50-96 h para Microsternarchus cf. bilineatus foi de 2,15 µg L-1, a menor concentração registrada para um peixe amazônico até o momento. Nenhuma das concentrações testadas deste inseticida afetou a atividade da AChE para o período de exposição testado. Um aumento significativo da atividade de GST no músculo foi detectado somente para as concentrações de 2 e 3 µg L-1. (AU)

Strain induced localized corrosion of NiTi, NiTiCo and NiTiCr alloys in 0.9% NaCl.

Shape memory and super elastic alloys are commonly used in biomedical and engineering areas, due to their higher elastic deformation characteristics and low elastic module when in martensitic state. For biomaterial applications, the alloy must exhibit adequate corrosion resistance and biocompatibility, especially in chloride environments. The addition of ternary elements in NiTi alloys aim to improve the mechanical properties. Addition of Co increases the elastic limit and reduce the transformation temperature while Cr additions increase the yield strength. However, it was demonstrated that this modification can affect the corrosion resistance of the raw materials. This study aims to assess the corrosion and strain induced corrosion resistance of NiTi alloys modified by Co and Cr additions in the presence of 0.9% NaCl solution. Ternary alloys were compared to NiTi binary alloys, when unstrained and strained within the elastic regime where martensitic transformation is induced. Electrochemical impedance spectroscopy (EIS) and anodic polarization tests were performed on both conditions. Straining electrode corrosion tests were performed under constant electrochemical potential being the electrochemical response registered. Tests using wire samples as straining working electrodes permitted the assessment of the correlation between deformation and the anodic current of the alloys immersed in 0.9% NaCl solution. It was concluded that, despite the mechanical benefits provided by the addition of ternary elements, these additions increased the susceptibility to localized corrosion and the pitting corrosion susceptibility enhanced by stress and corresponding strain.

Common Evaluations of Disease Activity in Rheumatoid Arthritis Reach Discordant Classifications across Different Populations.

OBJECTIVES: The classification of disease activity states in rheumatoid arthritis (RA) can be achieved through disease activity indices, such as the Disease Activity Score in 28 joints erythrocyte sedimentation rate (DAS28-ESR), the Simplified Disease Activity Index (SDAI), and the Clinical Disease Activity Index (CDAI). Subjective measurements, such as patient reported outcomes have been incorporated into several of these indices alongside more objective assessments, such as increases in the ESR and C-reactive protein. Moreover, while they use similar criteria, different indices weight these criteria to different extents. Therefore, the classifications based on each evaluation may not always be the same. We aim to compare the performance of the three indices and their individual components in two different populations. METHODS: Data from Dutch and Portuguese adherent centers were extracted from the METEOR database, a multinational collaboration on RA. We included a total of 24,605 visits from Dutch centers (from 5,870 patients) and 20,120 visits from Portuguese centers (from 3,185 patients). We compared the disease activity states as evaluated by the DAS28-ESR, CDAI, and SDAI across the two populations. In addition, we analyzed the individual components of each evaluation, including their respective contributions to the outcome, in each population. RESULTS: We found significant differences in the disease activity states classified with the DAS28-ESR between the two populations. SDAI and CDAI had more congruous results. While the proportion of visits to Dutch and Portuguese centers that were classified as "in remission" was very similar between the CDAI and SDAI, the DAS28-ESR gave discordant results. Dutch patients had lower ESRs, which is more heavily weighted in the DAS28-ESR. In addition, even though the mean physicians' global assessment values did not vary significantly for Dutch vs Portuguese physicians, we found that doctors at Portuguese centers overall scored the physician's global assessment lower than Dutch physicians for patient visits classified by disease activity state. CONCLUSION: While the CDAI and SDAI assigned disease activity states that were largely similar, the DAS28-ESR was often discordant across the two populations. Moreover, we found that physicians, more than patients, evaluated disease activity differently among the Portuguese and Dutch populations.

The Portuguese version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Portuguese language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, and construct validity (convergent and discriminant validity). A total of 80 JIA patients (6.3% systemic, 68.8% oligoarticular, 3.7% RF negative polyarthritis, 21.2% other categories) and 30 healthy children were enrolled. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. Notably, there was no significant difference between healthy subjects and their affected peers in school-related items. In conclusion, the Portuguese version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Effects of Neuroactive Drugs in the Discharge Patterns of Microsternarchus (Hypopomidae: Gymnotiformes) Electric Organ.

Considering the conserved nature of synaptic physiology among vertebrates, we tested the effects of three psychotropics (diazepam, doxapram, and nicotine) on Microsternarchus cf. bilineatus, measuring 10 parameters associated to the electric organ discharges rhythm and waveform before and after the administration of each drug and a control group. There were statistically significant differences (p < 0.005) among all the experimental groups, F (70, 22619.25) = 77.7, between the two experimental phases within their respective drug treatment, F (80, 24604.51) = 16.0, and among the six experimental hours within their respective phases and groups, F (320, 37124.15) = 4.1. We observed a common general trend of reduction in the electric organ's (EO) firing rate, regardless of the expected stimulant or depressor effect of the drugs on the central nervous system (CNS). The intensity of the response changed with the treatment. The observed changes in the fishes' behavior may be a result of the drugs' direct action on the CNS or a combination of this with systemic effects of each substance tested, also in the EO.

Reuma.pt contribution to the knowledge of immune-mediated systemic rheumatic diseases.

Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making. Being aware of the importance of registries, the Portuguese Society of Rheumatology developed the Rheumatic Diseases Portuguese Register- Reuma.pt - which proved to be an innovative instrument essential to a better understanding of systemic immune-mediated rheumatic diseases. OBJECTIVE: To describe the contribution of Reuma.pt to the knowledge of systemic immune-mediated rheumatic diseases. RESULTS: Reuma.pt is widely implemented, with 77 centres actively contributing to the recruitment and follow-up of patients. Reuma.pt follows in a standardized way patients with the following systemic inflammatory rheumatic diseases: rheumatoid arthritis (n=6218), psoriatic arthritis (n=1498), spondyloarthritis (n=2529), juvenile idiopathic arthritis (n =1561), autoinflammatory syndromes (n=122), systemic lupus erythematosus (n =1718), systemic sclerosis (n=180) and vasculitis (n=221). This platform is intended for use as an electronic medical record, provides standardized assessment of patients and support to the clinical decision, thereby contributing to a better quality of care of rheumatic patients. The research based on Reuma.pt identified genetic determinants of susceptibility and response to therapy, characterized in detail systemic rheumatic diseases and their long-term impact, critically appraised the performance of instruments for monitoring the disease activity, established the effectiveness and safety of biologic therapies and identified predictors of response, and proactively engaged patients in the management of their disease. CONCLUSION: Reuma.pt is an innovative tool, widely established in the country that contributes to a clinical practice of excellence and simultaneously to increase the knowledge of systemic immune-mediated rheumatic diseases. Additionally, Reuma.pt fosters patients' participation in the management of the disease.

Portuguese recommendations for the use of methotrexate in rheumatic diseases - 2016 update.

BACKGROUND: Methotrexate (MTX) is the first-line drug in the treatment of rheumatoid arthritis (RA) and the most commonly prescribed disease modifying anti-rheumatic drug. Moreover, it is also used as an adjuvant drug in patients under biologic therapies, enhancing the efficacy of biologic agents. OBJECTIVES: To review the literature and update the Portuguese recommendations for the use of MTX in rheumatic diseases first published in 2009. METHODS: The first Portuguese guidelines for the use of MTX in rheumatic diseases were published in 2009 and were integrated in the multinational 3E Initiative (Evidence Expertise Exchange) project. The Portuguese rheumatologists based on literature evidence and consensus opinion formulated 13 recommendations. At a national meeting, the recommendations included in this document were further discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the updated recommendations. RESULTS: Results presented in this article are mainly in accordance with previous guidelines, with some new information regarding hepatitis B infection during MTX treatment, pulmonary toxicity monitoring, hepatotoxicity management, association with hematologic neoplasms, combination therapy and tuberculosis screening during treatment. CONCLUSION: The present recommendations combine scientific evidence with expert opinion and attained desirable agreement among Portuguese rheumatologists. The regular update of these recommendations is essential in order to keep them a valid and useful tool in daily practice.

First Cytogenetic Description of Microsternarchus bilineatus (Gymnotiformes: Hypopomidae) from Negro River (Brazilian Amazon).

Microsternarchus bilineatus is a neotropical electric fish species widely distributed in Amazonian ecosystems. This work reports the first karyotypic description of the species M. bilineatus, which presented 2n = 48 chromosomes, with a distinct karyotypic formula between the sexes: males with 21 metacentric (m)/submetacentric (sm) + 27 subtelocentric (st)/acrocentric (a) and fundamental number (FN) = 69 and females with 20 m/sm + 28 st/a and FN = 68. We found a probable recent sex system of XX/XY type. The nucleoli organizer regions (NORs) were multiple terminally located, and the heterochromatic blocks were mostly pericentromeric. The 18S rDNA markings confirmed NORs and their distinction between sexes, which suggested some differential role of this gene related to gender in this species. The 5S rDNA presented terminal markings on a single chromosome pair, with no distinction between sexes, and the telomeric probes have shown a uniform pattern in males and females.

Comparative Effectiveness of Tocilizumab and TNF Inhibitors in Rheumatoid Arthritis Patients: Data from the Rheumatic Diseases Portuguese Register, Reuma.pt.

OBJECTIVES: To compare the effectiveness of TNF inhibitors (TNFi) and tocilizumab in rheumatoid arthritis (RA) treatment, according to different response criteria. METHODS: We included RA patients registered in the Rheumatic Diseases Portuguese Register treated with TNFi or tocilizumab for at least 6 months, between January 2008 and July 2013. We assessed remission/low disease activity (LDA) at 6 months according to DAS28, CDAI, and SDAI, as well as Boolean ACR/EULAR remission and EULAR response rate, adjusting for measured confounders. RESULTS: Tocilizumab-treated patients (n = 95) presented higher baseline disease activity and were less frequently naïve to biologics compared to TNFi users (n = 429). Multivariate logistic regression analysis including the propensity score for receiving tocilizumab showed that patients treated with tocilizumab were more likely to achieve remission or LDA according to DAS28 (OR = 11.0/6.2, 95% CI 5.6-21.6/3.2-12.0), CDAI (OR = 2.8/2.6, 95% CI 1.2-6.5/1.3-5.5), or SDAI (OR = 3.6/2.5, 95% CI 1.5-8.7/1.1-5.5), as well as a good EULAR response (OR = 6.4, 95% CI 3.4-12.0). However, both groups did not differ in Boolean remission (OR = 1.9, 95% CI 0.8-4.8) or good/moderate EULAR response (OR = 1.8, 95% CI 0.8-4.5). CONCLUSIONS: Compared with TNFi, tocilizumab was associated with greater likelihood of achieving DAS28, CDAI, and SDAI remission/LDA and EULAR good response. Boolean remission and EULAR good/moderate response did not differ significantly between groups.

DAS28, CDAI and SDAI cut-offs do not translate the same information: results from the Rheumatic Diseases Portuguese Register Reuma.pt.

OBJECTIVES: . The 28-joint DAS (DAS28), clinical disease activity index (CDAI) and simplified disease activity index (SDAI) are indices frequently used to assess disease activity in RA patients. Cut-off values were defined to classify the states of RA disease activity: remission, low, moderate and high. The aim of this work was to assess disease activity states classified by DAS28, CDAI and SDAI and to analyse their agreement in the Rheumatic Diseases Portuguese Register Reuma.pt. METHODS: . A total of 2795 patients and 14 440 visits were selected from Reuma.pt for analysis. Pearson's correlation coefficients (PCCs) were calculated for the three indices. McNemar's chi-squared tests, PCCs and kappa statistics were performed to analyse and compare the distribution of visits among all disease activity states and indices. RESULTS: A strong correlation was found between the three indices throughout the 14 440 visits: r = 0.874 for DAS28/CDAI, r = 0.877 for DAS28/SDAI and r = 0.984 for CDAI/SDAI (all PCCs with P < 0.0001). However, when categorization in the different disease activity states was analysed, McNemar's chi-squared tests and PCCs revealed significant disagreement between the cut-offs of the three indices. CONCLUSION: DAS28, CDAI and SDAI cut-offs do not translate into the same clinical information in Reuma.pt. Although this might be expected for the original DAS28 cut-offs, when compared with CDAI and SDAI significant disagreement was also found for the DAS28 modified cut-offs. For visits where patients are in CDAI or SDAI remission, we also find disagreement between these two indices, which may contradict previous conclusions that acute phase reactants add little to composite disease activity indices for RA.