Concomitant pulmonary embolism and aortic dissection: approach to anticoagulation

Os autores descrevem um caso de uma doente admitida após episódio pré-sincopal associado a precordialgia e cujo ecocardiograma sumário feito no Servic¸o de Urgência demonstrou alterac¸ões compatíveis com tromboembolismo pulmonar, tendo a doente sido submetida a terapêutica fibrinolítica, após episódio de paragem cardiorrespiratória. Na angiotomografia de tórax feita posteriormente para confirmac¸ão diagnóstica demonstra-se a presenc¸a não só de trombos no nível da artéria pulmonar, mas também de dissec¸ão da aorta Stanford B, tendo-se optado pela manutenc¸ão de anticoagulac¸ão terapêutica e a doente evoluído de forma favorável.The authors describe a case of a patient admitted with a pre-syncopal episode and precordial discomfort, and whose cardiac ultrasound performed in the Emergency Room was suggestive of Pulmonary Embolism. The patient was submitted to fibrinolytic therapy after cardiac arrest. The computerized tomography done after this episode not only confirmed the presence of pulmonary embolism but had also shown a Stanford Type B Aortic Dissection. The option was to maintain the therapeutic anticoagulation, having the patient evolved favourably.info:eu-repo/semantics/publishedVersio

Cuidados Paliativos Pediátricos: Relatório do Grupo de Trabalho do Gabinete do Secretário de Estado Adjunto do Ministro da Saúde

A visão dos Cuidados Paliativos Pediátricos é assegurar que desde o diagnóstico todos os utentes em idade pediátrica (recém-nascidos, crianças e jovens) que vivem com uma doença crónica complexa, limitante ou ameaçadora da vida, e suas famílias, recebam cuidados que vão ao encontro das suas necessidades, desejos e preferências, até e para além da morte. Os princípios consensuais para a provisão de cuidados de qualidade são 1) adesão à definição da Organização Mundial de Saúde, 2) centro na criança-família, 3) prestação no local preferido, 4) prestação baseada nas necessidades, desde o diagnóstico/reconhecimento e até depois da morte, 5) partilha de decisão entre criança-família-profissionais, 6) profissionais com formação, treino e experiência pediátricas, 7) equipas interdisciplinares, 8) redes integradas de serviços, 9) gestor de caso, 10) descanso do cuidador e 11) suporte permanente. Reconhecem-se três níveis na prestação de Cuidados Paliativos Pediátricos: nível 1 (básico – todos os profissionais que trabalham com crianças), nível 2 (generalista – profissionais que trabalham a tempo parcial em Cuidados Paliativos Pediátricos) e nível 3 (especialista – profissionais que trabalham exclusivamente em Cuidados Paliativos Pediátricos). Os serviços que venham a ser desenvolvidos devem ser integrados na prestação de cuidados de saúde, sustentáveis, apropriados à idade dos utentes e equitativos (independentes da idade, diagnóstico, local de residência, cultura ou condição socioeconómica da família). É importante realçar que as necessidades paliativas em Pediatria podem iniciar-se ainda antes do nascimento, impondo um acompanhamento especializado da família prestado em articulação entre a Obstetrícia e a Neonatologia. No outro extremo, a atual sobrevivência até à idade adulta de muitas crianças com patologias pediátricas raras exige a implementação de programas de transição para cuidados de saúde de adultos, incluindo Cuidados Paliativos. Ao mesmo tempo que se planeiam serviços deve existir uma preocupação com a formação e treino dos profissionais, único garante de cuidados de qualidade mesmo quando os recursos físicos não são os ideais. Esta formação não pode restringir-se aos aspetos médicos (controlo de sintomas), devendo abarcar igualmente as áreas da ética, comunicação e apoio no luto. Podemos estimar que vivam em Portugal pelo menos 6.000 crianças com necessidades paliativas; destas, cerca de 200 crianças morrem anualmente, quase todas no hospital (em 2011 apenas 1 em cada 9 morreu em casa). Os seus cuidados têm-se caraterizado por um foco excessivo na intervenção médica, centralização em hospitais terciários, fragmentação e ausência de coordenação, escassez de apoio domiciliário e psicossocial. Existem no entanto algumas experiências (de articulação, de reorganização de serviços, de apoio domiciliário) que se têm revelado sustentáveis e frutíferas, e que interessa agora replicar de forma organizada. Com base nas recomendações e experiências internacionais e na realidade nacional, o modelo de governação de Cuidados Paliativos Pediátricos mais indicado para Portugal baseia-se em quatro pilares: 1) formação de todos os profissionais envolvidos nos cuidados de crianças com necessidades paliativas, 2) promoção da prestação de cuidados domiciliários, 3) reorganização das instalações pediátricas existentes e 4) articulação eficaz entre todos os prestadores de cuidados de saúde (hospitalares e primários: Pediatria, Cuidados Paliativos e Medicina Geral e Familiar), rentabilizando os recursos humanos. Devem ser criadas equipas intrahospitalares pediátricas (nível 2/3) em todos os Departamentos de Pediatria, de recursos e níveis adequados às necessidades locais. Nos cinco maiores Centros Hospitalares o objetivo deve ser o nível 3 e a constituição de centros de referência regionais. Devem ser criadas unidades pediátricas que respondam de forma atempada e adequada às necessidades de internamento para capacitação ou descanso do cuidador, controlo de sintomas e fim de vida, numa lógica de proximidade e de continuidade de cuidados. Estimamos a necessidade de 60 camas a nível nacional, sendo preferível, pelo menos numa primeira fase, a sua criação junto ou incluídas nos Departamentos de Pediatria. Cada ACES deve dispor de pelo menos um pediatra e constituir um Núcleo para coordenação de todos os programas pediátricos existentes nos cuidados de saúde primários e comunidade. Este deve ser o responsável dentro do ACES e servir como interlocutor com os cuidados hospitalares. Devem ser acionadas redes funcionais regionais, com base na Rede Social e no Conselho da Comunidade dos ACES. Deve ser assegurada uma liderança estratégica através da criação de um Conselho Técnico- Científico, com a missão de constituir uma interface entre todos os decisores envolvidos, elaborar instrumentos para uso nacional, assegurar a qualidade das equipas, unidades e formações, promover a colaboração nacional e internacional e a educação e sensibilização dos decisores e do público em geral. Cremos ser possível concretizar estes objetivos a médio prazo sem custos excessivos para o país (sendo o principal investimento na formação de profissionais e expansão de equipas domiciliárias), desde que existam diretivas superiores e coordenação como as que podem ser proporcionadas pela criação de um Programa Nacional

Composite Outcomes in Observational Studies of Ulcerative Colitis: a Systematic Review and Meta‐Analysis

Background: Ulcerative colitis (UC) has been the focus of numerous observational studies over the years and a common strategy employed in their design is the use of composite and aggregate outcomes. Objective: This systematic review and meta-analysis aims to identify composite and aggregate outcomes of observational studies in UC and to evaluate how the number and type of variables included and the length of follow-up affect the frequency of patients that achieve these outcomes. Methods: A systematic literature search was carried out using MEDLINE [via PubMed], Scopus, and Web of Science online databases. Observational studies that included UC patients and reported composite or aggregate outcomes were identified. A set of variables considered to be representative of progressive or disabling UC was defined, the proportion of patients attaining the outcomes was determined and a random-effects meta-analysis was performed by dividing the identified studies into subgroups according to different criteria of interest. Results: A total of 10,264 records were identified in the systematic search, of which 33 were retained for qualitative analysis and 20 were included in the meta-analysis. The mean frequency for composite outcomes was 0.363 [95% confidence interval (CI) 0.323-0.403]. The frequency of composite outcome for the subgroup of studies that included the variable "Biologics" was significantly higher than for those in which this variable was not reported [0.410; 95% CI 0.364-0.457 versus 0.298; 95% CI 0.232-0.364; p = 0.006]. Composite outcomes were also more frequent as the follow-up duration increased. Conclusion: The frequency of composite outcomes in observational studies of UC is dependent on the specific identity of the variables being reported. Moreover, longer follow-up periods are associated with higher frequencies of composite outcomes. The evidence provided here is useful for the design of future observational studies of UC that aim to maximize the frequency of patients that achieve composite outcomes.info:eu-repo/semantics/publishedVersio

Crohn's disease in a southern European country: Montreal classification and clinical activity

BACKGROUND: Given the heterogeneous nature of Crohn's disease (CD), our aim was to apply the Montreal Classification to a large cohort of Portuguese patients with CD in order to identify potential predictive regarding the need for medical and/or surgical treatment. METHODS: A cross-sectional study was used based on data from an on-line registry of patients with CD. RESULTS: Of the 1692 patients with 5 or more years of disease, 747 (44%) were male and 945 (56%) female. On multivariate analysis the A2 group was an independent risk factor of the need for steroids (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.1-2.3) and the A1 and A2 groups for immunosuppressants (OR 2.2; CI 1.2-3.8; OR 1.4; CI 1.0-2.0, respectively). An L3+L3(4) and L(4) location were risk factors for immunosuppression (OR 1.9; CI 1.5-2.4), whereas an L1 location was significantly associated with the need for abdominal surgery (P < 0.001). After 20 years of disease, less than 10% of patients persisted without steroids, immunosuppression, or surgery. The Montreal Classification allowed us to identify different groups of disease severity: A1 were more immunosuppressed without surgery, most of A2 patients were submitted to surgery, and 52% of L1+L1(4) patients were operated without immunosuppressants. CONCLUSIONS: Stratifying patients according to the Montreal Classification may prove useful in identifying different phenotypes with different therapies and severity. Most of our patients have severe disease

Ulcerative colitis in a Southern European country: a national perspective

BACKGROUND: The incidence, prevalence, and even the clinical behavior of ulcerative colitis (UC) are highly variable in different world regions. In previous studies, Portugal was reported as having a milder clinical behavior. The aim of this study was to apply the Montreal Classification in a large group of UC Portuguese patients in order to describe their clinical characteristics and evaluate variables potentially useful for outcome prediction. METHODS: A cross-sectional study based on data collected from a nationwide online registry was undertaken. RESULTS: In all, 2863 patients with UC were included. Twenty-one percent had ulcerative proctitis, 52% left-sided colitis, and 28% extensive colitis. Sixty percent of patients had taken steroids, 14% immunosuppressors, 1% biologicals, and 4.5% were submitted to surgery. Patients with extensive colitis had more severe activity, needing more steroids, immunosuppressors, and surgery. At the time of diagnosis 61% were less than 40 years old and 5% less than 16. Younger patients also had a more aggressive initial course. Thirty-eight percent of patients had only taken salicylates during the disease course and were characterized by a lower incidence of systemic symptoms at presentation (3.8% versus 8.8%, P < 0.001), fewer extraintestinal manifestations (7.7% versus 24.0%, P < 0.001), and a higher prevalence of proctitis (32.1% versus 10.0%). CONCLUSIONS: A more aggressive phenotype was found in extensive colitis and in the initial course of younger patients, with an increased need for steroids and immunosuppressors. In addition, a significant percentage of patients, particularly with proctitis, showed a milder clinical evolution and were maintained in remission only with salicylates

Impact assessment of the European Clinical Trials Directive: a longitudinal, prospective, observational study analyzing patterns and trends in clinical drug trial applications submitted since 2001 to regulatory agencies in six EU countries

<p>Abstract</p> <p>Background</p> <p>Shifts in clinical trial application rates over time indicate if the attractiveness of a country or region for the conduct of clinical trials is growing or decreasing. The purpose of this observational study was to track changes in drug trial application patterns across several EU countries in order to analyze the medium-term impact of the EU Clinical Trials Directive 2001/20/EC on the conduct of drug trials.</p> <p>Methods</p> <p>Rates of Clinical Trial Applications (CTA) for studies with medicinal products in those six countries in the EU, which authorize on average more than 500 trials per year, were analyzed. Publicly available figures on the number of annually submitted CTA, the distribution of trials per phase and the type of sponsorship were tracked; missing data were provided by national drug agencies.</p> <p>Results</p> <p>Since 2001, the number of CTA in Italy and Spain increased significantly (5.0 and 2.5% average annual growth). For Italy, the gain was driven by a strong increase of applications from academic trial sponsors; Spain's growth was due to a rise in trials run by commercial sponsors. The Netherlands, Germany, France and the UK saw a decline (1.9, 2.3, 3.0 and 5.3% average annual diminution; significant (<it>P </it>< 0.05) except for Germany) in clinical drug trials. The decrease in the UK was caused by a sharp fall in academic trial activities. Across the six analyzed countries, no EU-wide trial-phase-specific patterns or trends were observed.</p> <p>Conclusions</p> <p>The EU Clinical Trials Directive 2001/20/EC did not achieve the harmonization of clinical trial requirements across Europe. Rather, it resulted in the leveling of clinical trial activities caused by a continuing decrease in CTA rates in the Netherlands, Germany, France and the UK. Southern European countries, Italy and Spain, benefited to some extent from policy changes introduced by the Directive. In Italy's case, national funding measures helped to considerably promote the conduct of non-commercial trials. On the other hand, the EU Directive-driven transition from liberal policy environments, based on non-explicit trial approval through notifications, towards red-taped processes of trial authorization, contributed to the decreases in trial numbers in Germany and the UK. In the latter case, national research governance concerns had a share in the country's marked decline. However, different EU member states successfully developed best practices, which a new European legislation should take into consideration to resume Europe's attractiveness and international competitiveness for the conduct of clinical trials.</p

Transmural Remission Improves Clinical Outcomes Up to 5 years in Crohn's Disease

Introduction: Evidence supporting transmural remission (TR) as a long-term treatment target in Crohn's disease (CD) is still unavailable. Less stringent but more reachable targets such as isolated endoscopic (IER) or radiologic remission (IRR) may also be acceptable options in the long-term. Methods: Multicenter retrospective study including 404 CD patients evaluated by magnetic resonance enterography and colonoscopy. Five-year rates of hospitalization, surgery, use of steroids, and treatment escalation were compared between patients with TR, IER, IRR, and no remission (NR). Results: 20.8% of CD patients presented TR, 23.3% IER, 13.6% IRR and 42.3% NR. TR was associated with lower risk of hospitalization (odds-ratio [OR] 0.244 [0.111-0.538], p < 0.001), surgery (OR 0.132 [0.030-0.585], p = 0.008), steroid use (OR 0.283 [0.159-0.505], p < 0.001), and treatment escalation (OR 0.088 [0.044-0.176], p < 0.001) compared to no NR. IRR resulted in lower risk of hospitalization (OR 0.333 [0.143-0.777], p = 0.011) and treatment escalation (OR 0.260 [0.125-0.540], p < 0.001), while IER reduced the risk of steroid use (OR 0.442 [0.262-0.745], p = 0.002) and treatment escalation (OR 0.490 [0.259-0.925], p = 0.028) compared to NR. Conclusions: TR improved clinical outcomes over 5 years of follow-up in CD patients. Distinct but significant benefits were seen with IER and IRR. This suggests that both endoscopic and radiologic remission should be part of the treatment targets of CD.info:eu-repo/semantics/publishedVersio

Accuracy of faecal calprotectin and neutrophil Gelatinase B-associated Lipocalin in evaluating subclinical inflammation in UlceRaTIVE colitis-the ACERTIVE study

Background and Aims: Mucosal healing and histological remission are different targets for patients with ulcerative colitis, but both rely on an invasive endoscopic procedure. This study aimed to assess faecal calprotectin and neutrophil gelatinase B-associated lipocalin as biomarkers for disease activity in asymptomatic ulcerative colitis patients. Methods: This was a multicentric cross-sectional study including 371 patients, who were classified according to their endoscopic and histological scores. These results were evaluated alongside the faecal levels of both biomarkers. Results: Macroscopic lesions [i.e. endoscopic Mayo score >= 1] were present in 28% of the patients, and 9% had active disease according to fht Ulcerative Colitis Endoscopic Index of Severity. Moreover, 21% presented with histological inflammation according to the Geboes index, whereas 15% and 5% presented with focal and diffuse basal plasmacytosis, respectively. The faecal levels of calprotectin and neutrophil gelatinase B-associated lipocalin were statistically higher for patients with endoscopic lesions and histological activity. A receiver operating characteristic-based analysis revealed that both biomarkers were able to indicate mucosal healing and histological remission with an acceptable probability, and cut-off levels of 150-250 mu g/g for faecal calprotectin and 12 mu g/g for neutrophil gelatinase B-associated lipocalin were proposed. Conclusions: Faecal calprotectin and neutrophil gelatinase B-associated lipocalin levels are a valuable addition for assessment of disease activity in asymptomatic ulcerative colitis patients. Biological levels of the analysed biomarkers below the proposed thresholds can rule out the presence of macroscopic and microscopic lesions with a probability of 75-93%. However, caution should be applied whenever interpreting positive results, as these biomarkers present consistently low positive predictive values.Portuguese IBD Group [GEDII - Grupo de Estudo da Doenca Inflamatcria Intestinal]info:eu-repo/semantics/publishedVersio