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Desafios na Abordagem do Enfarte com Elevação do Segmento ST nas Mulheres: um Olhar Atento
No full textBalloon Atrioseptostomy for Transposition of the Great Arteries in Europe: Characteristics and Outcomes.
No full textIntroduction and objectives: Balloon atrial septostomy (BAS) improves oxygenation in neonates with transposition of the great arteries (TGA) and restrictive foramen ovale. Currently, there is a global shortage of dedicated BAS catheters, while new unmarked catheters have recently become available at some European centers. This study aimed to characterize BAS outcomes using the currently available BAS catheters in Europe.
Methods: A 2-year multicenter observational registry was conducted, including all neonates undergoing BAS for TGA. We report preliminary results (September 2022-February 2024) focusing on BAS characteristics and outcomes.
Results: A total of 250 BAS procedures were performed in 29 centers. The median neonatal weight was 3.16kg, and 88% of neonates had a prenatal diagnosis. Most procedures were performed often on the first day of life during working hours (72.8%), mainly in catheterization laboratories (59.2%). Guidance primarily involved ultrasound with or without fluoroscopy. A guidewire was used in 41.2% of procedures. A total of 290 catheters (286 Z-5 or Z-6) were used, achieving an overall BAS success rate of 96%. Complete procedural failure was associated with the use of the umbilical venous route (OR, 3.62; P=.001) and lower-volume catheters (OR, 7.01; P<.001). The occurrence of significant complications (8%; OR, 9.33; P<.001) was associated with complete procedural failure. For complex procedures, significant risk factors were the absence of fluoroscopy (OR, 3.32; P=.001), use of the umbilical venous route (OR, 2.28; P=.005), and lower-volume catheters (OR, 2.43; P=.03).
Conclusions: In the current era, BAS can be challenging, and significant complications and complete failures are not uncommon. The use of the umbilical venous route, low-volume BAS catheters, absence of fluoroscopy guidance, and the occurrence of complications negatively impact procedural outcomes
Tintelnotia Destructans, the Rare Opportunist of a Behçet's Disease Patient.
No full textBehçet's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers that can have ophthalmologic, cutaneous, neurologic, vascular, and thromboembolic manifestations. Treatment usually involves immunosuppressant medication, which leads to an increased risk of opportunistic infections. Only recently identified, is a rare fungus that can cause eye and nail infections in humans, usually refractory to standard antifungal therapy. Ocular infections are most commonly associated with ocular trauma or the use of contact lenses and may cause permanent damage without adequate treatment. We present a case of a 40-year-old man with Behçet's disease, treated with adalimumab, who developed an ocular abscess due to . This clinical case serves the purpose of alerting for an opportunistic infection caused by a newly described and rare microorganism, which is hard to identify
Hipoperfusão Abdominal e Lesão Renal Aguda no Doente Crítico com Cirrose Hepática – Estudo de Coorte Prospetivo
No full textReduced abdominal perfusion pressure (APP) is an underdiagnosed potential pathophysiological mechanism for acute kidney injury (AKI) in the patient with liver cirrhosis and ascites. This study aimed to analyze the prevalence of abdominal hypoperfusion (AhP) (APP <60 mm Hg) and the impact of APP on AKI in critically ill patients with liver cirrhosis.
This was a post hoc analysis from a prospective cohort study set in a general ICU at a tertiary university hospital. Patients were recruited between October 2016 and December 2021. Acute renal failure (ARF) was defined by stage 3 AKI according to the International Club of Ascites.
Fifty-eight patients where included, with a mean age of 57 (±8.4) years, 79% were male, and 93% had acute-on-chronic liver failure at admission. The prevalence of AhP reached 75%, and 29% of cases had persisting AhP during the first week of ICU stay. Patients with baseline AhP had a higher 28-day mortality compared to those without AhP (respectively, 76% vs. 49%, = 0.03). Acute renal failure developed in 48% of patients. Higher serum urea (aOR: 1.01, 95% CI: 1.00-1.02, = 0.04) and white blood cell count (aOR: 1.1, 95% CI: 1.01-1.2, = 0.02) at ICU admission, as well as low persisting APP (aOR: 0.9, 95% CI: 0.86-0.98, = 0.02) were independent risk factors for ARF.
Critically ill patients with liver cirrhosis presented a high prevalence of ARF, independently associated with higher baseline serum urea and WBC, and lower persisting APP. A structured clinical approach to optimize APP may reduce renal dysfunction in high-risk patients with cirrhosis.A pressão de perfusão abdominal (PPA) é um possível mecanismo fisiopatológico para a lesão renal aguda (LRA) frequentemente sub-diagnosticado no paciente cirrótico com ascite. Este estudo teve como objetivo analisar a prevalência de hipoperfusão abdominal (hPA) (PPA <60 mm Hg) e o impacto da PPA na lesão renal aguda em doentes com cirrose e doença crítica.
Esta foi uma análise pós-hoc de um estudo de coorte prospetivo de doentes críticos com cirrose hepática realizado numa unidade de cuidados intensivos (UCI) polivalente de um hospital universitário terciário. Os doentes foram recrutados entre outubro de 2016 e dezembro de 2021. A falência renal aguda (FRA) foi definida de acordo com o estadio 3 de LRA do International Club of Ascites.
Cinquenta e oito doentes foram incluídos, com uma média de idade de 57 (±8.4) anos, 79.3% eram do sexo masculino e 93.1% apresentavam a síndrome . A prevalência de hPA foi de 75.3%, e 29.3% dos casos apresentaram hPA persistente durante a primeira semana na UCI. Os doentes com hPA basal apresentaram um aumento de mortalidade aos 28 dias em comparação com aqueles sem hPA (76.0% vs. 48.5%, = 0.03). Verificou-se FRA na admissão em 48.3% dos pacientes. O aumento da concentração da ureia sérica (aOR 1.01, IC95% 1.001–1.02, = 0.04) e da contagem de leucócitos (CL) (aOR 1.1, 95% IC: 1.01–1.2, = 0.02) na admissão à UCI, bem como a redução persistente da PPA (aOR 0.9, 95% IC: 0.86–0.98, = 0.02) foram fatores de risco independentes para o desenvolvimento de FRA.
Os doentes críticos com cirrose hepática apresentaram uma alta prevalência de FRA, cujos fatores de risco independentes incluíram o aumento da ureia sérica e da CL basais, e a redução persistente da PPA. Uma abordagem clínica estruturada para otimizar a PPA poderá reduzir a lesão renal aguda nos doentes cirróticos de alto risco
Diagnosis and Predictors of Post-Implantation Syndrome Following Endovascular Repair of Aortic Aneurysms – a Narrative Review
No full textINTRODUCTION: After endovascular aortic repair (EVAR), many patients develop a systemic inflammatory response called post-implantation syndrome (PIS). AAA and procedure-related characteristics have been linked with increased odds of developing this syndrome. Similarly, some short- and long-term consequences have been associated with PIS. This study aims to review the literature on the diagnosis and predictors of post-implantation after endovascular repair of aortic aneurysms.
RESULTS: A non-systematic review of the MEDLINE and Scopus databases was performed using the keywords "abdominal aortic aneurysm," "inflammation," and "endovascular techniques.” No time or language limitations were imposed. Manuscripts were considered irrespective of study design. Articles of interest were analyzed, and the relevant information was organized in tables.
RESULTS: PIS is defined as a combination of constitutional symptoms, including fatigue and fever, and elevated inflammatory markers. There are several proposed diagnostic criteria, most including a combination of fever with leukocytosis and/or elevated C-reactive protein (CRP). These result in discrepant rates, as low as 2% and up to 100%. The typical evolution of this syndrome is spontaneous resolution, although pharmacologic measures for symptom relief may be needed. These symptoms often resolve within two weeks; no significant permanent complications remain. Most PIS cases will present up to the first 72 postoperative hours. Endograft material, particularly polyester-based stent grafts, has been consistently linked to increased odds of PIS, up to five-fold, compared to polytetrafluoroethylene (PTFE) grafts. Aneurysm thrombus load (both pre-existing and new-onset) has also been related to an increased odds of PIS. Bacterial translocation, contrast media, and other patient or procedure-related characteristics have not been linked to an increased risk of PIS.
CONCLUSION: PIS is a common finding after EVAR. Universal diagnostic criteria for diagnosis are required. Polyester-based stent grafts present the highest risk of developing this syndrome. Aneurysm thrombus load may also relate to this increased risk. The impact of other clinical or anatomical factors remains undetermined
COVID- 19 in Patients Affected by Red Blood Cell Disorders, Results From the European Registry ERN-EuroBloodNet.
No full textBackground: Despite several publications covering patients from multiple centers, no international registry covered all patients with red blood cell diseases (RBCD) affected by COVID- 19. The ERN-EuroBloodNet's registry provided real-time registration of SARS-CoV- 2 patients with RBCD, promoting timely disease-specific knowledge sharing during the pandemic's early stages.
Procedures: The study evaluated patient distribution, the infection across different RBCDs, and severity risk factors across similar healthcare systems, using data collected from the ERN-EuroBloodNet's REDCap platform.
Results: From April 2020 to April 2023, 681 infections were recorded among 663 patients, of which 373 had transfusion-dependent thalassemia or non-transfusion-dependent thalassemia (TDT/NTDT), and 269 had sickle cell disease (SCD). SCD patients had a higher incidence of COVID- 19 than those with TDT/NTDT (10.5 vs. 4.8 COVID/100 patients). Notably, 92% of the cases were mild, with neither age nor the specific RBCD affecting severity. The number of comorbidities, notably obesity and hypertension, that patients had prior to infection was associated with more severe COVID- 19. During the infection, the presence of vaso-occlusive crises, acute chest syndrome, kidney failure, and ground-glass opacities on chest tomography scans were associated with a more severe clinical picture. The vaccination rate (32%) mirrored that of the general population and showed a protective effect against severe COVID- 19. The observed mortality rate was 0.7%, aligning with Europe's general population.
Conclusion: SARS-CoV- 2 infection in SCD and TDT/NTDT patients is mild and without higher mortality than the general population. The ERN-Eurobloodnet's registry collaborative structure exemplifies the power of international cooperation in tackling rare diseases, especially during health emergencies
Analysis of Stereotyped B-Cell Receptor Frequencies Among Portuguese De Novo-Diagnosed Chronic Lymphocytic Leukemia Patients (PAIS Study).
No full textProtocol 54179060CLL4020/Johnson & Johnson (Portugal)Background/objectives: Chronic lymphocytic leukemia (CLL) exhibits a heterogeneous clinical course influenced by genetic factors, such as the mutational status of immunoglobulin variable regions (IGHV). Recently, B-cell receptor (BcR) stereotypes have shown promising prognostic value, potentially surpassing IGHV status. The PAIS study analyzed BcR stereotypes and IGHV mutations in newly diagnosed Portuguese CLL patients to assess prognostic characteristics and disease progression.
Methods: This cross-sectional study included 463 adult patients from 15 Portuguese centers, recruited between November 2020 and September 2023. The median age at diagnosis was 70.4 years. The most common clinical stages were 0 (54%) and 1 (32.83%).
Results: A total of 15 different BcR stereotypes were identified in the cohort studied. Subtype #1, associated with a poorer prognosis, was the most prevalent, observed in 3.90% of newly diagnosed Portuguese CLL patients. Considering the 19 major stereotypes that could be assigned by the ARResT subsets tool, most patients exhibited a heterogeneous BcR profile (90.14%). A total of 57.24% of patients had mutated IGHV. The concentration of β2-microglobulin was significantly lower in patients with mutated IGHV (2.6 mg/L vs. 3.6 mg/L, p < 0.001). Clinical stage, assessed by the RAI staging system, differed between subgroups, with a higher frequency of stage 0 in patients with mutated IGHV and stage 2 in unmutated patients (p = 0.009).
Conclusions: The PAIS study highlighted the predominance of a heterogeneous BcR profile in Portuguese CLL patients. The higher percentage of patients with mutated IGHV at diagnosis supports prior findings. This study improves the characterization of the 10% of Portuguese CLL patients with major BcR stereotypes, offering healthcare providers better predictive power for disease progression and potentially impacting clinical decision making
Carbapenem-Resistant Enterobacteriaceae Colonization or Infection Was Not Associated with Post-Liver Transplant Graft Failure: an Observational Cohort Study.
No full textIntroduction: Carbapenem-resistant Enterobacteriaceae (CRE) epidemiology among liver transplant (LT) recipients is variable. We studied the impact of CRE colonization and infection on LT recipients' outcomes.
Methods: This observational cohort study included consecutive adult LT recipients between January 2019 and December 2020 at Curry Cabral Hospital, Lisbon, Portugal. Primary exposures were CRE colonization (rectal swabs under a screening program) and infection within 1 year of index LT. Primary endpoint was graft failure within 1 year of the index LT.
Results: Among 209 patients, the median (interquartile range [IQR]) age was 57 (47-64) years and 155 (74.2%) were male. CRE colonization was identified in 28 (13.4%) patients during the first year posttransplant (median [IQR] number of rectal swabs per patient of 4 [2-7]). CRE resistance genes identified were OXA48 in 8 (3.6%) patients, KPC in 19 (67.9%) patients, and VIM in 1 (3.6%) patient. Any bacterial/fungal and CRE infections were diagnosed in 88 (42.1%) and 6 (2.9%) patients, respectively, during the first year posttransplant. After adjusting for confounders, neither CRE colonization (aOR [95% CI] = 1.83 [0.71-4.70]; p = 0.21) nor infection (aOR [95% CI] = 1.35 [0.17-11.06]; p = 0.78) was associated with graft failure within 1 year of index LT.
Discussion/conclusion: Under a screening program, CRE colonization and infection prevalence was low and neither was associated with graft failure
[Costs and Consequences of Chronic Kidney Disease in People with Diabetes in Portugal: A Modelling Study].
No full textIntroduction: Chronic kidney disease is the fastest-growing chronic disease in terms of prevalence and one of the biggest causes of global mortality according to the Global Burden of Disease Collaboration. This study aimed to project the natural disease progression of this disease in people with diabetes, and to quantify the costs and consequences in the Portuguese context. This was achieved by developing an analytical model reflecting the epidemiology of chronic kidney disease and integrating the various stages of disease progression.
Methods: A population-based cohort Markov model was used, to follow an adult cohort of people with diabetes and chronic kidney disease as they progressed through different risk categories, in annual cycles, over a period of 50 years. The model considered the natural progression of chronic kidney disease through 18 risk categories based on the KDIGO classification system, as well as the probability of patients receiving renal replacement therapy, including dialysis and kidney transplantation, and the probability of death. Each stage is associated with an annual cost and a disability weight, so the model allowed survival, years lived with disability and lifetime costs to be estimated for the entire population with chronic kidney disease and for patients in different risk categories.
Results: Over the cohort´s lifetime, the model estimated, for the total population with chronic kidney disease and diabetes, an average survival of 8.62 years, with 0.59 years lived with disability, and an average cost of €24 613. These figures correspond to a loss of more than 410 000 years lived with disability and a total lifetime cost of 17.0 billion euros. The progression of this disease was associated with lower survival, more years lived with disability and higher costs.
Conclusion: The results of this study characterize the natural progression of chronic kidney disease in people with diabetes mellitus type 2, as well as the associated costs and consequences in the national context. Since diabetes mellitus type 2 is a risk factor for chronic kidney disease, it is expected that the real impact will be greater than estimated in the coming decades. Analysis by risk level shows that progression of the disease is associated with worse outcomes.Introdução: A doença renal crónica é a doença crónica com maior crescimento de prevalência, e uma das maiores causas de mortalidade global de acordo com o Global Burden of Disease Collaboration. O presente estudo teve como objetivo projetar a evolução desta doença em pessoas com diabetes, de modo a quantificar os custos e consequências no contexto português. Tal foi conseguido através do desenvolvimento e parametrização de um modelo analítico refletindo a epidemiologia da doença renal crónica e integrando os vários estádios de progressão da doença. Métodos: Foi utilizado um modelo populacional de coorte com uma mecânica de Markov, onde pessoas com diabetes e doença renal crónica foram seguidas ao longo de 50 anos, em ciclos anuais, sendo registada a sua progressão através das diferentes categorias de risco da doença renal crónica. O modelo considerou a progressão natural da doença renal crónica através de 18 categorias de risco baseados na matriz de estadiamento de KDIGO, bem como a probabilidade de os doentes receberem terapêutica de substituição renal, designadamente, diálise e transplantação renal e a probabilidade de morte. A cada estádio estão associados um custo anual e um ponderador de incapacidade, pelo que o modelo permitiu estimar a sobrevivência, os anos de vida perdidos por incapacidade (years lived with disability), e os custos incorridos ao longo da vida, para a totalidade da população e para doentes em diferentes categorias de risco. Resultados: Durante o tempo total de evolução da coorte, o modelo estimou, para a população total com doença renal crónica e diabetes, uma sobrevivência média de 8,62 anos, com 0,59 anos perdidos por incapacidade, e um custo médio lifetime de €24 613. Estes valores correspondem a mais de 410 mil anos de vida perdidos por incapacidade e um custo total, ao longo da vida, de 17,0 mil milhões de euros. A análise por nível de risco demonstra que a progressão da doença renal crónica está associada a menor sobrevivência, mais anos perdidos por incapacidade e maiores custos. Conclusão: Os resultados deste estudo caracterizam a progressão natural da doença renal crónica em pessoas com diabetes mellitus tipo 2 bem como os custos e consequências associados no contexto nacional. Sendo a diabetes mellitus tipo 2 um fator de risco da doença renal crónica, é expectável que nas próximas décadas o impacto real seja maior do que o estimado. A análise por nível de risco permite verificar que a progressão da doença está associada a piores resultados
The Microevolutionary Trajectory of Endemic Multidrug-Resistant Tuberculosis Strains in Portugal Toward Increased Drug Resistance Levels and its Clinical Significance.
No full textPortugal has one of the highest incidence rates of tuberculosis (TB) in Western Europe and, historically, multidrug-resistant (MDR) cases have been strongly associated with strains pertaining to the endemic Q1 and Lisboa3 clades. Notwithstanding, the contribution of drug resistance-associated allelic configurations in these clades to differing levels of drug resistance and their relationship with drug efficacy has yet to be uncovered. A representative sample of the drug-resistant population in Portugal, comprised of 40 clinical strains were subjected to whole genome sequencing for characterization of allelic combinations of drug resistance-associated mutations and their minimum inhibitory concentrations for 12 anti-TB drugs was determined. Pharmacokinetic (PK) models were generated to ascertain the maximum concentration to which each drug remains efficacious. Drug resistance levels were determined and compared between different allelic configurations. Double and mutation genotypes contributed with increased isoniazid and ethambutol resistance levels compared with single mutation configurations, respectively. Significant differences in drug resistance levels were observed between phylogenetic groups for rifamycin, streptomycin and ethionamide, largely explained by the presence/absence of unique high-level resistance-associated genotypes. The PK models for isoniazid and moxifloxacin suggest an increase in dosage to be ineffective against strains harboring high-level resistance-conferring double mutations and mutations. Cycloserine and para-aminosalicylic acid are the only drugs predicted to remain efficacious against the majority of tested strains, while the effectiveness of newer drugs like bedaquiline, pretomanid and delamanid have yet to be uncovered. Proper diagnosis of drug resistance-associated mutations provides invaluable insights into the treatment of TB, as different allelic configurations lead to differing drug resistance levels, often rendering drugs ineffective