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- 2023 ACR/EULAR antiphospholipid syndrome classification criteriaPublication . Barbhaiya, Medha; Zuily, Stephane; Naden, Ray; Hendry, Alison; Manneville, Florian; Amigo, Mary-Carmen; Amoura, Zahir; Andrade, Danieli; Andreoli, Laura; Artim-Esen, Bahar; Atsumi, Tatsuya; Avcin, Tadej; Belmont, Michael H; Bertolaccini, Maria Laura; Branch, D Ware; Carvalheiras, Graziela; Casini, Alessandro; Cervera, Ricard; Cohen, Hannah; Costedoat-Chalumeau, Nathalie; Crowther, Mark; de Jesús, Guilherme; Delluc, Aurelien; Desai, Sheetal; Sancho, Maria De; Devreese, Katrien M; Diz-Kucukkaya, Reyhan; Duarte-García, Ali; Frances, Camille; Garcia, David; Gris, Jean-Christophe; Jordan, Natasha; Leaf, Rebecca K; Kello, Nina; Knight, Jason S; Laskin, Carl; Lee, Alfred I; Legault, Kimberly; Levine, Steve R; Levy, Roger A; Limper, Maarten; Lockshin, Michael D; Mayer-Pickel, Karoline; Musial, Jack; Meroni, Pier Luigi; Orsolini, Giovanni; Ortel, Thomas L; Pengo, Vittorio; Petri, Michelle; Pons-Estel, Guillermo; Gomez-Puerta, Jose A; Raimboug, Quentin; Roubey, Robert; Sanna, Giovanni; Seshan, Surya V; Sciascia, Savino; Tektonidou, Maria G; Tincani, Angela; Wahl, Denis; Willis, Rohan; Yelnik, Cécile; Zuily, Catherine; Guillemin, Francis; Costenbader, Karen; Erkan, DorukObjective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR. Methods: This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard. Results: The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β2-glycoprotein I antibodies). Patients accumulating at least three points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria vs the 2006 revised Sapporo classification criteria had a specificity of 99% vs 86%, and a sensitivity of 84% vs 99%. Conclusion: These new ACR/EULAR APS classification criteria were developed using rigorous methodology with multidisciplinary international input. Hierarchically clustered, weighted, and risk-stratified criteria reflect the current thinking about APS, providing high specificity and a strong foundation for future APS research.
- ABCESSOS PULMONARES: REVISÃO DE 60 CASOSPublication . Magalhães, L.; Valadares, D.; Oliveira, J.; Reis, E.
- Acute Heart Failure Comorbidome: The Impact of Everything ElsePublication . Meireles, Mariana; Gonçalves, João; Neves, JoãoIntroduction: Heart failure frequently coexists with several comorbidities. Our aim is to evaluate the prognostic role of various comorbidities in the risk of acute heart failure development. Material and methods: Comorbidities of patients with acute heart failure were, retrospectively, compared to a control group of patients with chronic heart failure admitted to an Internal Medicine unit in a 2-year period. Logistic regression models were constructed to determine their association with acute heart failure and to develop a comorbidome. Results: We identified 229 patients with acute heart failure and 201 patients with chronic heart failure. Age and female gender were higher in acute heart failure group (p < 0.001) as was the number of comorbidities (4.0 ± 3.0 vs 4.0 ± 2.0, p = 0.044). Hyperuricemia (odds ratio 2.46, confidence interval 95% 1.41 - 4.31, p = 0.002), obesity (odds ratio 2.22, confidence interval 95% 1.31 - 3.76, p = 0.003), atrial fibrillation (odds ratio 1.93, confidence interval 95% 1.31 - 2.87, p = 0.001), peripheral artery disease (odds ratio 2.12, confidence interval 95% 1.01 - 4.42, p = 0.046) and chronic kidney disease (odds ratio 2.47, confidence interval 95% 1.65 - 3.71, p < 0.001) were associated with acute heart failure. Obesity, atrial fibrillation, peripheral artery disease and chronic kidney disease were identified as independent risk factors. Patients with multiple comorbidities had a superior risk of hospitalization due to heart failure: zero comorbidities - odds ratio 0.43, 95% confidence interval 0.28 - 0.67, p < 0.001; one comorbidity - odds ratio 0.69, 95% confidence interval 0.47 - 1.01, p = 0.057; two comorbidities - odds ratio 1.85, 95% confidence interval 1.11 - 3.08, p = 0.019; ≥ three comorbidities - odds ratio 5.81, 95% confidence interval 2.77 - 12.16, p < 0.001. Discussion: This study shows an association between several comorbidities and hospital admission due to acute heart failure. The association seems to strengthen in the presence of multiple comorbidities. Conclusion: A comorbidome is a useful tool to identify comorbidities associated with higher risk of acute heart failure. The identification of vulnerable patients may allow multidimensional interventions to minimize future hospital admissions.
- Adult Native Joint Septic Arthritis: A Nine-Year Retrospective Analysis in a Portuguese University HospitalPublication . Cipriano, Ana; Videira Santos, Fábio; Dias, Rita; Carvalho, André; Reis, Ernestina; Pereira, Claudia; Santos, Ana Cláudia; Sousa, Ricardo; Abreu, Miguelntroduction: Septic arthritis of a native joint represents a medical emergency. Drainage and effective antibiotic treatment are critical to avoid joint destruction and long-term impairment. The aim of this study was to evaluate epidemiological and clinical characteristics of patients with the diagnosis of septic arthritis to help establish local guidelines for empirical antibiotic treatment. Material and methods: Retrospective analysis of adult patients admitted at Centro Hospitalar Universitário do Porto from 2009 to 2017 with suspected native joint septic arthritis. Relevant demographics, microbiology findings and respective antibiotic susceptibilities were analysed. Results: Ninety-seven patients, predominantly males (59.8%) with a median age of 61 years old were included. The most commonly reported comorbidity associated with septic arthritis was diabetes mellitus (20.6%). The knee was the most commonly affected joint (71.1%). Arthrocentesis was performed in all patients, but only 50.5% had positive microbial growth in the synovial fluid. Staphylococcus aureus was the most frequently identified microorganism, 86% of which were methicillin susceptible. Gram-negative bacteria were the causative agent in 15% of cases. A wide range of empirical antibiotic regimens were prescribed with a combination of vancomycin/carbapenem being the most common (30.9%). Analysis of antibiotic susceptibility profiles revealed that amoxicillin/clavulanate would have been appropriate as the initial regimen in 89% of cases. Discussion: The main causative pathogen was Staphylococcus aureus, with methicillin resistant Staphylococcus aureus remaining rare. The proportion of Gram-negative bacteria implies that these agents should be covered by empirical treatment, although no case of Pseudomonas infection has been identified. Therefore, antipseudomonal coverage is not necessary in empirical regimens. Conclusion: Routine coverage of methicillin-resistant Staphylococcus aureus and Pseudomonas aeruginosa is not warranted but must be considered when specific risk factors are found. Amoxicillin/clavulanate can provide adequate antibiotic coverage as an empirical treatment for adult native joint septic arthritis. Its use may allow a reduction in use of broader spectrum antibiotics.
- Alteplase for Massive Pulmonary Embolism after Complicated PericardiocentesisPublication . Marinho, Ricardo Cleto; Martins, J.; Costa, S.; Baptista, R.; Gonçalves, L.; Franco, F.Background: The occurrence of a high-risk pulmonary embolism (PE) within 48 hours of a complicated pericardiocentesis to remove a haemorrhagic pericardial effusion, is an uncommon clinical challenge. Case summary: The authors report the case of a 75-year-old woman who presented with signs of imminent cardiac tamponade due to recurring idiopathic pericardial effusion. The patient underwent pericardiocentesis that was complicated by the loss of 1.5 litres of blood. Within 48 hours, the patient had collapsed with clear signs of obstructive shock. This was a life-threating situation so alteplase was administered after cardiac tamponade and hypertensive pneumothorax had been excluded. CT chest angiography later confirmed bilateral PE. The patient achieved haemodynamic stability less than an hour after receiving the alteplase. However, due to the high risk of bleeding, the medical team suspended the thrombolysis protocol and switched to unfractionated heparin within the hour. The cause of the PE was not identified despite extensive study, but after 1 year of follow-up the patient remained asymptomatic. Discussion: Despite the presence of a contraindication, the use of thrombolytic therapy in obstructive shock after exclusion of hypertensive pneumothorax can be life-saving, and low-dose thrombolytic therapy may be a valid option in such cases. Learning points: A quick and systematic approach to a collapsed patient with signs of shock is mandatory; understanding the type of shock may help narrow the differential diagnosis and help in therapeutic decisions.After exclusion of cardiac tamponade and hypertensive pneumothorax, life-saving thrombolytic therapy can be administered in obstructive shock due to probable massive pulmonary embolism.Contraindications for thrombolytic therapy originated as exclusion criteria for clinical trials but should not prevent the use of this therapy in life-threatening situations.
- An Immunological Non-responder Human Immunodeficiency Virus/Hepatitis C Virus Coinfected Patient: Considerations About a Clinical CasePublication . Correia, Rui; França, MargaridaHuman immunodeficiency virus (HIV) and hepatitis C virus (HCV) infections are two chronic viral infections that share the same mode of transmission, making HIV/HCV coinfection frequent. Highly active antiretroviral therapy (HAART) was a turning point in HIV treatment and has been shown to successfully restore immune function and reduce the frequency of opportunistic infections. Despite a virological response to HAART, a proportion of patients fail to achieve substantial immune recovery, as measured by peripheral CD4 cell counts. Herein, we present the case of a patient with HIV/HCV coinfection who did not achieve successful immune function restoration despite HIV suppression and HCV treatment. Our goal is to promote discussion. Despite considerable advances in the understanding of the impact of HCV on HIV disease progression, there are many individual variables that influence a patient's immune function. In addition, we consider hypogammaglobulinemia as a possible contributor. Further understanding and improvement of immune reconstitution in patients infected with HIV remain an important field of scientific research.
- ANCA-positive vasculitis: Clinical implications of ANCA types and titersPublication . Domingues, V.; Machado, B.; Santos, J.INTRODUCTION: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease that can affect multiple organs, the kidney being one of the most affected. Apart from the diagnostics value of ANCA, they have also been advocated as biomarkers of the disease activity. Recently, the genetic changes found in polyangiitis associated with serine-protease proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA raised the possibility of immune-pathogenic and therapeutic differences. OBJECTIVE: To identify differences in the number of relapses, inflammatory markers, outcomes and renal histology related to the types of ANCA. To analyze the implications of ANCA titers in prognosis. METHOD: A retrospective observational study in a Portuguese tertiary hospital. RESULTS: There were no differences in the progression of renal function, histological pattern and initial treatment with regard to ANCA subtypes. As for the evaluated parameters, there were no significant differences according to the types of ANCA, except for mean CRP values within the normal range, which was 6.3±1.3 mg/L for MPO-ANCA and 12.4±10.14 mg/L for PR3-ANCA (p=0.04). We found that 66.7% of the MPO-ANCA-positive showed no relapses versus 40% in the case of PR3-ANCA-positive. There was no correlation between the ANCA titers at presentation, during remission, and in the last evaluation, and the number of relapses. CONCLUSION: PR3-ANCA patients have a mean CRP value within the normal range significantly higher than that of MPO-ANCA patients (p=0.04), which seems to reveal greater inflammatory activity in the first.
- Anti-Ro52 Antibodies and Interstitial Lung Disease in ConnectiveTissue Diseases Excluding SclerodermaPublication . Ferreira, J.; Almeida, I.; Marinho, A.; Cerveira, C.; Vasconcelos, C.The presence of anti-Ro52 antibodies has been reported in a wide variety of autoimmune diseases, particularly in myositis, scleroderma, and autoimmune liver diseases. Clinical significance of anti-Ro52 antibodies remains controversial, and studies are lacking for clarifying the association of anti-Ro52 with interstitial lung disease (ILD) in connective tissue diseases (CTD). Objectives. To determine if anti-Ro52 antibodies are associated with ILD in CTD other than scleroderma. Methods. Singlecenter, retrospective study based on immunoblotting panel analysis and patients clinical records. Results. In our connective tissue disease cohort, 162 patients had immunoblotting panels with anti-Ro52 reactivity analysis, 41 (25,3%) had inclusion criteria. Among the 41 selected sera, 85.4% (n = 35) had anti-Ro52 reactivity. The prevalence of ILD in the positive anti-Ro52 antibodies was 71.4% (n = 25), and 16.7% (n = 1) in the negative anti-Ro52 group (P = 0.018). Overall sensitivity (96.2%), specificity (83.3%), positive (71.4%) and negative (83.3%) predictive values of anti-Ro52 antibodies to determine ILD in CTD is detailed in this study. Conclusion. Ro52 autoantibodies are associated with ILD in CTD excluding scleroderma. We suggest that the presence of anti-Ro52 reactivity in CTD should increase the clinician curiosity for the search of ILD.
- Antineutrophil cytoplasmatic antibody positive systemic vasculitis in a patient treated with propylthiouracilPublication . Silva, S.; Ferreira, J.; Carvalho, S.; Seabra, F.; Marinho, A.
- Aortic Dissection With Cardiac Tamponade in Pregnancy: A Challenging Clinical ScenarioPublication . Barroso, Daniela; Santos, Sérgio; Tomás, Ana Sofia; Castro, Heloísa; Pinheiro Vieira, AntónioAortic dissection is the acute aortic syndrome with the highest mortality, and pregnancy and arterial hypertension are known risk factors. Its association with the perinatal period is a particularly unique and potentially devastating clinical catastrophe which is why the approach to a pregnant woman in cardiorespiratory arrest (CRA) should be multidisciplinary and early, with extraction of the fetus ideally within five minutes after the arrest. We present the case of a 39-year-old pregnant woman, who presented with a cardiorespiratory arrest in the context of an aortic dissection with cardiac tamponade and the need for an urgent perimortem cesarean section. Increasing knowledge and understanding among healthcare professionals has the potential to aid in the early detection and effective treatment of this challenging medical issue.