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1.
Clin Neuropharmacol ; 46(3): 105-111, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37191564

RESUMO

OBJECTIVES: Cladribine is a selective and oral immunological reconstitution treatment, approved in Europe for very active multiple sclerosis (MS) with relapses. Aims were to assess the safety and effectiveness of cladribine in real-world setting, during treatment follow-up. METHODS: This was a multicentric, longitudinal, observational study with retrospective and prospective data collection of clinical, laboratory, and imaging data. This interim analysis reports data from July 1, 2018 (study onset), to March 31, 2021. RESULTS: A total of 182 patients were enrolled: 68.7% were female; mean age at onset was 30.1 ± 10.0 years, and mean age at first cycle of cladribine treatment was 41.1 ± 12.1; 88.5% were diagnosed with relapse-remitting MS and 11.5% with secondary progressive MS. Mean disease duration at cladribine start was 8.9 ± 7.7 years. Most patients (86.1%) were not naive, and median number of previous disease-modifying therapies was 2 (interquartile range, 1-3). At 12 months, we observed no significant Expanded Disability Status Scale score worsening ( P = 0.843, Mann-Whitney U test) and a significantly lower annualized relapse rate (0.9 at baseline to 0.2; 78% reduction). Cladribine treatment discontinuation was registered in 8% of patients, mainly (69.2%) due to disease activity persistence. Most frequent adverse reactions were lymphocytopenia (55%), infections (25.2%), and fatigue (10.7%). Serious adverse effects were reported in 3.3%. No patient has discontinued cladribine treatment because of adverse effects. CONCLUSION: Our study confirms the clinical efficacy and the safety profile of cladribine for treating MS patients with a long-term active disease in the real-world setting. Our data contribute to the body of knowledge of the clinical management of MS patients and the improvement of related clinical outcomes.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Humanos , Feminino , Masculino , Cladribina/efeitos adversos , Esclerose Múltipla/tratamento farmacológico , Imunossupressores/efeitos adversos , Portugal/epidemiologia , Estudos Retrospectivos , Centros de Atenção Terciária , Recidiva , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico
2.
J Card Fail ; 29(4): 517-526, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36632933

RESUMO

Heart failure (HF) is a clinical syndrome that is divided into 3 subtypes based on the left ventricular ejection fraction. Every subtype has specific clinical characteristics and concomitant diseases, substantially increasing risk of thromboembolic complications, such as stroke, peripheral embolism and pulmonary embolism. Despite the annual prevalence of 1% and devastating clinical consequences, thromboembolic complications are not typically recognized as the leading problem in patients with HF, representing an underappreciated clinical challenge. Although the currently available data do not support routine anticoagulation in patients with HF and sinus rhythm, initial reports suggest that such strategy might be beneficial in a subset of patients at especially high thromboembolic risk. Considering the existing evidence gap, we aimed to review the currently available data regarding coagulation disorders in acute and chronic HF based on the insight from preclinical and clinical studies, to summarize the evidence regarding anticoagulation in HF in special-case scenarios and to outline future research directions so as to establish the optimal patient-tailored strategies for antiplatelet and anticoagulant therapy in HF. In summary, we highlight the top 10 pearls in the management of patients with HF and no other specific indications for oral anticoagulation therapy. Further studies are urgently needed to shed light on the pathophysiological role of platelet activation in HF and to evaluate whether antiplatelet or antithrombotic therapy could be beneficial in patients with HF. LAY SUMMARY: Heart failure (HF) is a clinical syndrome divided into 3 subtypes on the basis of the left ventricular systolic function. Every subtype has specific clinical characteristics and concomitant diseases, substantially increasing the risk of thromboembolic complications, such as stroke, peripheral embolism and pulmonary embolism. Despite the annual prevalence of 1% and devastating clinical consequences, thromboembolic complications are not typically recognized as the leading problem in patients with HF, representing an underappreciated clinical challenge. Although the currently available data do not support routine anticoagulation in patients with HF and no atrial arrhythmia, initial reports suggest that such a strategy might be beneficial in a subset of patients at especially high risk of thrombotic complications. Considering the existing evidence gap, we aimed to review the currently available data regarding coagulation problems in stable and unstable patients with HF based on the insight from preclinical and clinical studies, to summarize the evidence regarding anticoagulation in HF in specific patient groups and to outline future research directions to establish the optimal strategies for antiplatelet and anticoagulant therapy in HF, tailored to the needs of an individual patient. In summary, we highlight the top 10 pearls in the management of patients with HF and no other specific indications for oral anticoagulation therapy.


Assuntos
Fibrilação Atrial , Transtornos da Coagulação Sanguínea , Insuficiência Cardíaca , Embolia Pulmonar , Acidente Vascular Cerebral , Tromboembolia , Humanos , Volume Sistólico , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/epidemiologia , Função Ventricular Esquerda , Anticoagulantes/uso terapêutico , Tromboembolia/tratamento farmacológico , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Acidente Vascular Cerebral/etiologia , Transtornos da Coagulação Sanguínea/complicações , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Arritmias Cardíacas , Fibrilação Atrial/complicações
3.
Lisboa; s.n; 2023.
Tese em Português | BDENF - Enfermagem | ID: biblio-1509392

RESUMO

Assiste-se a uma transformação sociodemográfica mundial caracterizada pelo rápido envelhecimento populacional. Paralelamente, com o aumento da acessibilidade à medicação antirretroviral de alta eficácia, a esperança média de vida das pessoas que vivem com VIH aumentou. Pela primeira vez observa-se um maior número de pessoas com 50 ou mais anos que vivem com VIH. Estas pessoas têm maior prevalência de comorbilidade, síndromes geriátricos e estigma traduzidos em menor qualidade de vida. Embora as necessidades sejam complexas, os sistemas de saúde ainda mantém uma abordagem episódica e centrada no controlo imunitário e virológico. Para dar resposta às necessidades das pessoas idosas que vivem com VIH é necessária uma abordagem centrada na pessoa, implicando capacidade de resposta e sensibilidade às necessidades e preferências individuais, incluindo as específicas das populações-chave. Este relatório documenta as atividades desenvolvidas na unidade curricular de estágio com relatório do curso de mestrado em enfermagem, na área de especialização em enfermagem médico-cirúrgica, na área de intervenção de enfermagem à pessoa idosa da Escola Superior de Enfermagem de Lisboa. Foi utilizada a metodologia de projeto com base no quadro conceptual do Modelo de Cuidados Centrados na Pessoa proposto por McCormack e McCance (2006) com o objetivo de identificar as intervenções de enfermagem promotoras da gestão de saúde da pessoa idosa que vive com VIH. O percurso realizado contribuiu para o desenvolvimento de competências de mestre e enfermeiro especialista com especial enfoque na pessoa idosa que vive com VIH em diferentes contextos de prática clínica.


We are witnessing a global socio-demographic change characterized by rapid populational ageing. In parallel, with increased accessibility to highly active antiretroviral therapy, the life expectancy of people living with HIV has increased. For the first time there is a greater number of people 50 years of age and older living with HIV. These people have a higher prevalence of comorbidities, geriatric syndromes and stigma translated into lower quality of life. Although people needs are complex, health systems still maintain an episodic and immune/virologic focused approach. Addressing the needs of elderly people living with HIV requires a person-centred approach, implying responsiveness and sensitivity to individual needs and preferences, including those specific to key populations. This report documents the activities carried out in the curricular unit "internship with report" of the master's degree in nursing, in the area of specialization in medical-surgical nursing, in the area of nursing intervention for the elderly at the Nursing School of Lisbon. The methodology used was based on the conceptual Person-Centered Framework proposed by McCormack and McCance (2006), in order to identify nursing interventions that promote the health management of elderly people living with HIV. The internship contributed to the development of master's and specialist nurse competencies with special focus on the elderly person living with HIV in different contexts of clinical practice.


Assuntos
Idoso , Planejamento de Assistência ao Paciente , Idoso , Síndrome de Imunodeficiência Adquirida , Assistência Integral à Saúde , Gerenciamento Clínico , Enfermagem Geriátrica , Serviços de Saúde para Idosos
4.
Rev Port Cardiol ; 41(10): 887.e1-887.e5, 2022 10.
Artigo em Inglês, Português | MEDLINE | ID: mdl-36055898

RESUMO

Takotsubo syndrome (TTS) is currently described as an acute and usually reversible form of systolic dysfunction of the left ventricle, which more frequently affects postmenopausal women after a stressful emotional event. Although TTS is a rare condition in premenopausal women, in recent years, the number of reported cases has increased. This manuscript reports the first case of a TTS several months after delivery in a 22-year-old woman during lactation. It may also emphasize the role of estrogens in the disease pathogenesis.


Assuntos
Cardiomiopatias , Cardiomiopatia de Takotsubo , Adulto , Cardiomiopatias/complicações , Estrogênios , Feminino , Ventrículos do Coração , Humanos , Cardiomiopatia de Takotsubo/etiologia , Adulto Jovem
5.
Mult Scler Relat Disord ; 63: 103845, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35594635

RESUMO

INTRODUCTION: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. OBJECTIVE: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). METHODS: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. RESULTS: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). CONCLUSIONS: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.


Assuntos
Mielite Transversa , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Feminino , Humanos , Masculino , Neuromielite Óptica/epidemiologia , Portugal/epidemiologia
7.
Mult Scler Relat Disord ; 56: 103258, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34583213

RESUMO

INTRODUCTION: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. OBJECTIVE: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. METHODS: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. RESULTS: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. CONCLUSION: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries.


Assuntos
Neuromielite Óptica , Adulto , Aquaporina 4 , Autoanticorpos , Estudos Epidemiológicos , Feminino , Humanos , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/epidemiologia , Portugal/epidemiologia
8.
Mult Scler Relat Disord ; 55: 103176, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34343868

RESUMO

BACKGROUND: Therapeutic inertia (TI) is defined as a failure to initiate or intensify treatments despite evidence of disease activity. Its prevalence and determining factors in Relapsing-Remitting Multiple Sclerosis (RRMS) patients in Portugal are not known. The main objective of this work was to ascertain the prevalence of TI in RRMS and its determining factors. METHODS: We conducted a multicentre retrospective observational study of RRMS patients followed in MS Clinics of six Portuguese hospitals with at least one medical appointment in 2018. TI was defined as the absence of treatment initiation or intensification when therapeutic goals were unmet, that is when there was evidence of disease activity based on the definition of "no evidence of disease activity" (NEDA) which refers to absence of clinical relapses, absence of disease progression measured by expanded disability status scale (EDSS) and absence of new disease activity (new T2 lesions/enhancing lesion) on magnetic resonance imaging (MRI) over the period of observation. RESULTS: We included 427 patients with RRMS meeting inclusion criteria, 69.6% females, with a mean age of 41.66 years old. The mean age at diagnosis was 33.17 years old and the average number of years since diagnosis was 8.72. MS relapses were reported on 54 patients. Moderate to severe relapses were reported in 59.3%. Median EDSS score was 1.5. Intention to get pregnant was explicit in 39 patients, representing 18.8% of the women at childbearing age. Among the 365 patients who had an MRI, 23.8% had new T2 lesions and 7.4% had enhancing lesions. Regarding DMT, 72.8% were treated with interferon, glatiramer acetate, teriflunomide, or dimethyl fumarate, 20.6% were under fingolimod, natalizumab, rituximab, and cladribine, and the remaining 6.6% were without treatment. Adverse events were reported in 12.9% of patients, and 10.1% mentioned preferences regarding the treatment. TI was present in 80 (18.7%) patients, representing 54.8% of those with potential to inertia. Patients with a radiologically less active disease, who were already on a DMT and who had no adverse events from their current treatment were more likely to have TI (p<0,05). Also, patients followed in centers classified as higher level of care (level 1) had more TI compared with patients followed in centers of levels 2 and 3. CONCLUSION: TI was present in 1 in 5 patients, exceeding half of the sample with the potential to inertia, corroborating the high prevalence of TI in other studies. The determining factors of TI were the absence of relapses or the occurrence of mild relapses, being already on DMT, absence of adverse events, and follow-up in higher care level centers. TI is a topic rarely addressed in MS and this work highlights the importance of therapeutic optimization in these patients. Further studies should be held to explore the factors that influence TI once they have a great impact on therapeutic decisions.


Assuntos
Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Adulto , Feminino , Cloridrato de Fingolimode , Acetato de Glatiramer , Humanos , Masculino , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Natalizumab , Gravidez
9.
Blood Rev ; 50: 100864, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34217531

RESUMO

Sepsis is a complex syndrome with a high incidence, increasing by 8.7% annually over the last 20 years. Coagulopathy is a leading factor associated with mortality in patients with sepsis and range from slight thrombocytopenia to fatal disorders, such as disseminated intravascular coagulation (DIC). Platelet reactivity increases during sepsis but prospective trials of antiplatelet therapy during sepsis have been disappointing. Thrombocytopenia is a known predictor of worse prognosis during sepsis. The mechanisms underlying thrombocytopenia in sepsis have yet to be fully understood but likely involves decreased platelet production, platelet sequestration and increased consumption. DIC is an acquired thrombohemorrhagic syndrome, resulting in intravascular fibrin formation, microangiopathic thrombosis, and subsequent depletion of coagulation factors and platelets. DIC can be resolved with treatment of the underlying disorder, which is considered the cornerstone in the management of this syndrome. This review presents the current knowledge on the pathophysiology, diagnosis, and treatment of sepsis-associated coagulopathies.


Assuntos
Transtornos da Coagulação Sanguínea , Coagulação Intravascular Disseminada , Sepse , Trombocitopenia , Transtornos da Coagulação Sanguínea/diagnóstico , Transtornos da Coagulação Sanguínea/epidemiologia , Transtornos da Coagulação Sanguínea/etiologia , Coagulação Intravascular Disseminada/diagnóstico , Coagulação Intravascular Disseminada/epidemiologia , Coagulação Intravascular Disseminada/etiologia , Humanos , Estudos Prospectivos , Sepse/complicações , Sepse/diagnóstico
10.
J Orthop Case Rep ; 11(3): 13-15, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34239821

RESUMO

INTRODUCTION: A monoarthritic painful knee may have multiple etiologies, of which septic arthritis is commonly considered to be one of the most deleterious pathologies. This type of acute symptom is, in the literature, for some rare cases, related to an inflammatory process secondary to a penetrating plant thorn, which was considered an aseptic process and therefore treated by removing the foreign body. This work reports a recent case falling into that diagnosis, which due to its infrequent nature, could have been misevaluated and consequently mistreated. CASE REPORT: Our goal is to bring to the attention of the orthopedic community that this type of deleterious pathology, which is today uncommon, must never be fully disregarded. We share a case of a 68-year-old male who was admitted 2 days after being injured by a palm tree (Arecaceae) thorn with acute inflammatory signs and effusion in the left knee. Although, ultrasound excluded pathway or intra-articular foreign bodies, in the arthroscopic procedure, a thorn was found and removed. Pantoea agglomerans was identified in the synovial fluid. CONCLUSION: Following the removal of the foreign body, empirical antibiotic treatment with coverage against Gram-negative pathogens was initiated and later on replaced by appropriate antibiotics, after susceptibility testing is obtained.

11.
Front Neurol ; 12: 613769, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33790847

RESUMO

The spread of the COVID-19 pandemic has imposed significant challenges on healthcare provision, requiring changes in the conventional patient management, particularly in chronic diseases like multiple sclerosis (MS). To increase patient safety and reduce the risk of infection, while ensuring an appropriate and regular follow-up, tele-medicine gained prominence as a valid alternative to face-to-face appointments. However, the urgency of the implementation and the lack of experience in most MS centers led to "ad hoc" and extremely diverse approaches, which now merit to be standardized and refined. Indeed, while tele-consultation cannot fully replace face-to-face visits, it certainly can, and will, be incorporated as part of the routine care of MS patients in the near future. Bearing this in mind, the Portuguese Multiple Sclerosis Study Group (GEEM) has developed a set of recommendations for the usage of tele-medicine in the management of MS patients, both during the pandemic and in the future. The consensus was obtained through a two-step modified Delphi methodology, resulting in 15 recommendations, which are detailed in the manuscript.

12.
Mult Scler Relat Disord ; 51: 102865, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33714125

RESUMO

BACKGROUND: A significant proportion of pediatric-onset multiple sclerosis (POMS) patients do not respond to first-line disease-modifying therapies. Clinical trials showed that natalizumab is effective and safe in adults, but there are limited clinical trial data for children. Natalizumab is currently prescribed off-label for POMS. We aimed to characterize the effectiveness, safety and tolerability of natalizumab in all POMS cases treated in Portugal (from 2007 to 2018). METHODS: Data from clinical records were retrospectively collected for all POMS cases treated with natalizumab in Portugal. RESULTS: Twenty-one patients were included, 14 (67%) of which were female. The median age at POMS diagnosis was 13 years old. The median duration of treatment with natalizumab was 2 years and 3 months. Median Expanded Disability Status Scale score decreased from 1.5 to 1.0 after 24 months. The Annualized Relapse Rate decreased from 1.31 events/patient/year before treatment with natalizumab to 0 after 12 months of treatment and to 0.04 after 24 months. No gadolinium-enhancing lesions or new or enlarged T2 hyperintense lesions were observed in 8/8 patients (100%) after 12 months, and 4/5 (80%) after 24 months. There was one possible serious adverse event, which did not require dose adjustment. Five patients discontinued treatment due to positive anti-JCV (JC virus) antibody JC serostatus. CONCLUSION: Natalizumab may be an effective and safe disease-modifying therapy for POMS. Our results are in line with data published for the adult population, as well as with similar observational studies in pediatric populations in other regions.


Assuntos
Vírus JC , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Adolescente , Adulto , Criança , Feminino , Humanos , Fatores Imunológicos/efeitos adversos , Esclerose Múltipla/tratamento farmacológico , Natalizumab/efeitos adversos , Portugal , Estudos Retrospectivos
13.
Cardiovasc Drugs Ther ; 35(2): 215-229, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33074525

RESUMO

INTRODUCTION: Emerging evidence points to an association between severe clinical presentation of COVID-19 and increased risk of thromboembolism. One-third of patients hospitalized due to severe COVID-19 develops macrovascular thrombotic complications, including venous thromboembolism, myocardial injury/infarction and stroke. Concurrently, the autopsy series indicate multiorgan damage pattern consistent with microvascular injury. PROPHYLAXIS, DIAGNOSIS AND TREATMENT: COVID-19 associated coagulopathy has distinct features, including markedly elevated D-dimers concentration with nearly normal activated partial thromboplastin time, prothrombin time and platelet count. The diagnosis may be challenging due to overlapping features between pulmonary embolism and severe COVID-19 disease, such as dyspnoea, high concentration of D-dimers, right ventricle with dysfunction or enlargement, and acute respiratory distress syndrome. Both macro- and microvascular complications are associated with an increased risk of in-hospital mortality. Therefore, early recognition of coagulation abnormalities among hospitalized COVID-19 patients are critical measures to identify patients with poor prognosis, guide antithrombotic prophylaxis or treatment, and improve patients' clinical outcomes. RECOMMENDATIONS FOR CLINICIANS: Most of the guidelines and consensus documents published on behalf of professional societies focused on thrombosis and hemostasis advocate the use of anticoagulants in all patients hospitalized with COVID-19, as well as 2-6 weeks post hospital discharge in the absence of contraindications. However, since there is no guidance for deciding the intensity and duration of anticoagulation, the decision-making process should be made in individual-case basis. CONCLUSIONS: Here, we review the mechanistic relationships between inflammation and thrombosis, discuss the macrovascular and microvascular complications and summarize the prophylaxis, diagnosis and treatment of thromboembolism in patients affected by COVID-19.


Assuntos
Anticoagulantes/farmacologia , Coagulação Sanguínea , COVID-19 , Administração dos Cuidados ao Paciente/métodos , Trombose , Coagulação Sanguínea/efeitos dos fármacos , Coagulação Sanguínea/imunologia , Testes de Coagulação Sanguínea/métodos , COVID-19/sangue , COVID-19/imunologia , COVID-19/fisiopatologia , COVID-19/terapia , Humanos , Prognóstico , Trombose/etiologia , Trombose/fisiopatologia , Trombose/prevenção & controle , Trombose/terapia
14.
Am Heart J ; 226: 114-126, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32531501

RESUMO

BACKGROUND: Fabry disease (FD) is a treatable cause of hypertrophic cardiomyopathy (HCM). We aimed to determine the independent predictors of FD and to define a clinically useful strategy to discriminate FD among HCM. METHODS: Multicenter study including 780 patients with the ESC definition of HCM. FD screening was performed by enzymatic assay in males and genetic testing in females. Multivariate regression analysis identified independent predictors of FD in HCM. A discriminant function analysis defined a score based on the weighted combination of these predictors. RESULTS: FD was found in 37 of 780 patients with HCM (4.7%): 31 with p.F113L mutation due to a founder effect; and 6 with other variants (p.C94S; p.M96V; p.G183V; p.E203X; p.M290I; p.R356Q/p.G360R). FD prevalence in HCM adjusted for the founder effect was 0.9%. Symmetric HCM (OR 3.464, CI95% 1.151-10.430), basal inferolateral late gadolinium enhancement (LGE) (OR 10.677, CI95% 3.633-31.380), bifascicular block (OR 10.909, CI95% 2.377-50.059) and ST-segment depression (OR 4.401, CI95% 1.431-13.533) were independent predictors of FD in HCM. The score ID FABRY-HCM [-0.729 + (2.781xBifascicular block) + (0.590xST depression) + (0.831xSymmetric HCM) + (2.130xbasal inferolateral LGE)] had a negative predictive value of 95.8% for FD, with a cut-off of 1.0, meaning that, in the absence of both bifascicular block and basal inferolateral LGE, FD is a less probable cause of HCM, being more appropriate to perform HCM gene panel than targeted FD screening. CONCLUSION: FD prevalence in HCM was 0.9%. Bifascicular block and basal inferolateral LGE were the most powerful predictors of FD in HCM. In their absence, HCM gene panel is the most appropriate step in etiological study of HCM.


Assuntos
Cardiomiopatia Hipertrófica/etiologia , Doença de Fabry/complicações , Doença de Fabry/diagnóstico , Adulto , Idoso , Doença de Fabry/genética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Linhagem
15.
J Cardiovasc Imaging ; 28(2): 123-133, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32233165

RESUMO

BACKGROUND: Stress echocardiography has a 72%-85% sensitivity and an 80%-95% specificity. In this study, we characterized patients who received a false-positive stress echocardiogram result. METHODS: A total of 5,256 patients underwent a stress echocardiogram (induced by exercise, dobutamine, or dipyridamole) between 2009 to 2018, and 405 patients (7.7%) received a positive result. Among the positive patients, 300 underwent coronary angiography within 12 months, and these patients were included in this study (mean age = 64.9 ± 9.4 years, 230 men [76.7%]). Coronary artery disease was diagnosed by stenosis ≥50% in any epicardial coronary artery. Clinical and echocardiographic variables were compared between patients with true- and false-positive stress echocardiogram results. RESULTS: Seventy-two patients (24%) had a false-positive stress echocardiogram, with similar rates across stressor types (p = 0.574). Patients with false positives were less frequently men (63.9% vs. 80.7%, p = 0.003), had lower diabetes mellitus prevalence (15.3% vs. 45.6%, p = 0.001), were similar to true positive patients with regard to body-mass index, arterial hypertension prevalence, hyperlipidemia and smoking, and had lower pre-test probability of coronary artery disease (23% vs. 32%, p = 0.016). The wall motion score index (WMSI) was higher in the true-positive stress group, and wall motion abnormalities were more frequent in the apical segments (70.5% vs. 56.7%, p = 0.034). In a multivariable predictive model, men (odds ratio [OR] = 2.994), diabetes (OR = 5.440), and peak WMSI (OR = 10.690) were associated with a true-positive result. CONCLUSIONS: Twenty-four percent of our study population received a false-positive stress echocardiogram result, with similar rates across stressor types. Patients with true-positive stress echocardiogram results are more likely to be men, diabetic, and have a high peak WMSI.

16.
Arq. bras. cardiol ; 113(5): 948-957, Nov. 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1055042

RESUMO

Abstract Backgrund: New-onset atrial fibrillation complicating acute myocardial infarction represents an important challenge, with prognostic significance. Objective: To study the incidence, impact on therapy and mortality, and to identify predictors of development of new-onset atrial fibrillation during hospital stay for ST-segment elevation myocardial infarction. Methods: We studied all patients with ST-elevation myocardial infarction included consecutively, between 2010 and 2017, in a Portuguese national registry and compared two groups: 1 - no atrial fibrillation and 2 - new-onset atrial fibrillation. We adjusted a logistic regression model data analysis to assess the impact of new-onset atrial fibrillation on in-hospital mortality and to identify independent predictors of its development. A p value < 0.05 was considered significant. Results: We studied 6325 patients, and new-onset atrial fibrillation was found in 365 (5.8%). Reperfusion was successfully accomplished in both groups with no difference regarding type of reperfusion. In group 2, therapy with beta-blockers and angiotensin-conversion enzyme (ACE) inhibitors/angiotensin receptor blockers (ARBs) was less frequent, 20.6% received anticoagulation at discharge and 16.1% were on triple therapy. New-onset atrial fibrillation was associated with more in-hospital complications and mortality. However, it was not found as an independent predictor of in-hospital mortality. We identified age, prior stroke, inferior myocardial infarction and complete atrioventricular block as independent predictors of new-onset atrial fibrillation. Conclusion: New-onset atrial fibrillation remains a frequent complication of myocardial infarction and is associated with higher rate of complications and in-hospital mortality. Age, prior stroke, inferior myocardial infarction and complete atrioventricular block were independent predictors of new onset atrial fibrillation. Only 36.7% of the patients received anticoagulation at discharge.


Resumo Fundamento: A fibrilação auricular de novo no contexto de infarto agudo do miocárdio representa um importante desafio com potencial impacto prognóstico. Objetivo: Determinar a incidência, impacto na terapêutica e mortalidade, e identificar possíveis preditores do aparecimento de fibrilação auricular de novo durante o internamento por infarto agudo do miocárdio com supradesnivelamento do segmento ST. Métodos: Estudamos todos os pacientes com infarto agudo do miocárdio com supradesnivelamento do segmento ST inseridos consecutivamente de 2010 a 2017 num registro nacional português e comparamos dois grupos: 1 - sem fibrilação auricular; 2- com fibrilação auricular de novo. Efetuamos análise com modelo de regressão logística para avaliar o impacto de fibrilação auricular de novo na mortalidade intra-hospitalar e identificar preditores independentes para o seu aparecimento. Para teste de hipóteses, considerou-se significativo p < 0,05. Resultados: Estudamos 6325 pacientes, dos quais 365 (5.8%) apresentaram fibrilação auricular de novo. Não houve diferença no número de pacientes reperfundidos nem na estratégia de reperfusão. No grupo 2, terapêutica com betabloqueadores e IECA/ARA foi menos frequente, 20.6% tiveram alta sob anticoagulação oral e 16.1% sob terapêutica tripla. A fibrilação auricular de novo associou-se a maior incidência de complicações e mortalidade intra-hospitalar, mas não foi preditor independente de mortalidade intra-hospitalar. Identificamos idade, acidente vascular cerebral prévio, infarto inferior e bloqueio auriculoventricular completo como preditores independentes de fibrilação auricular de novo. Conclusões: A fibrilação auricular de novo continua sendo uma complicação frequente do infarto agudo do miocárdio, estando associada a aumento das complicações e mortalidade intra-hospitalar. Apenas 36.7% desses pacientes teve alta sob anticoagulação.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/complicações , Stents/estatística & dados numéricos , Infarto do Miocárdio com Supradesnível do Segmento ST/complicações , Portugal/epidemiologia , Recidiva , Fibrilação Atrial/mortalidade , Fibrilação Atrial/terapia , Fármacos Cardiovasculares/uso terapêutico , Reperfusão Miocárdica/mortalidade , Incidência , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores Etários , Mortalidade Hospitalar , Angiografia Coronária , Trombectomia/mortalidade , Acidente Vascular Cerebral/complicações , Infarto do Miocárdio com Supradesnível do Segmento ST/mortalidade , Infarto do Miocárdio com Supradesnível do Segmento ST/terapia , Insuficiência Cardíaca/complicações , Hospitalização/estatística & dados numéricos , Tempo de Internação
17.
Arq Bras Cardiol ; 113(5): 948-957, 2019 11.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31553385

RESUMO

BACKGRUND: New-onset atrial fibrillation complicating acute myocardial infarction represents an important challenge, with prognostic significance. OBJECTIVE: To study the incidence, impact on therapy and mortality, and to identify predictors of development of new-onset atrial fibrillation during hospital stay for ST-segment elevation myocardial infarction. METHODS: We studied all patients with ST-elevation myocardial infarction included consecutively, between 2010 and 2017, in a Portuguese national registry and compared two groups: 1 - no atrial fibrillation and 2 - new-onset atrial fibrillation. We adjusted a logistic regression model data analysis to assess the impact of new-onset atrial fibrillation on in-hospital mortality and to identify independent predictors of its development. A p value < 0.05 was considered significant. RESULTS: We studied 6325 patients, and new-onset atrial fibrillation was found in 365 (5.8%). Reperfusion was successfully accomplished in both groups with no difference regarding type of reperfusion. In group 2, therapy with beta-blockers and angiotensin-conversion enzyme (ACE) inhibitors/angiotensin receptor blockers (ARBs) was less frequent, 20.6% received anticoagulation at discharge and 16.1% were on triple therapy. New-onset atrial fibrillation was associated with more in-hospital complications and mortality. However, it was not found as an independent predictor of in-hospital mortality. We identified age, prior stroke, inferior myocardial infarction and complete atrioventricular block as independent predictors of new-onset atrial fibrillation. CONCLUSION: New-onset atrial fibrillation remains a frequent complication of myocardial infarction and is associated with higher rate of complications and in-hospital mortality. Age, prior stroke, inferior myocardial infarction and complete atrioventricular block were independent predictors of new onset atrial fibrillation. Only 36.7% of the patients received anticoagulation at discharge.


Assuntos
Fibrilação Atrial/complicações , Infarto do Miocárdio com Supradesnível do Segmento ST/complicações , Stents/estatística & dados numéricos , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/mortalidade , Fibrilação Atrial/terapia , Fármacos Cardiovasculares/uso terapêutico , Angiografia Coronária , Feminino , Insuficiência Cardíaca/complicações , Mortalidade Hospitalar , Hospitalização/estatística & dados numéricos , Humanos , Incidência , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Reperfusão Miocárdica/mortalidade , Portugal/epidemiologia , Valor Preditivo dos Testes , Recidiva , Estudos Retrospectivos , Infarto do Miocárdio com Supradesnível do Segmento ST/mortalidade , Infarto do Miocárdio com Supradesnível do Segmento ST/terapia , Acidente Vascular Cerebral/complicações , Volume Sistólico , Análise de Sobrevida , Trombectomia/mortalidade
18.
Rev Bras Ortop (Sao Paulo) ; 54(3): 316-321, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31363287

RESUMO

Objective Focal osteochondral lesions of the knee are found in two thirds of patients undergoing arthroscopy; their treatment, when isolated and especially in young individuals, remains a debating topic. The present study analyzes the results obtained by the application of the mosaicplasty technique on the treatment of isolated knee femoral condyle osteochondral lesions. Methods Retrospective study of patients submitted to mosaicplasty and to subjective analyses with pre- and postsurgery International Knee Documentation Committee (IKDC) scores. Results A total of 13 cases with an average age of 34 years old, with male patients ( n = 4; 31%) with an average age of 23 years old (range: 17-31 years old), and female patients ( n = 9; 69%) with an average age of 39 years old (range: 16-56 years old); medial versus lateral femoral ( n = 11; 85% versus n = 2; 15%); the average size of the lesion was 1.8 cm 2 (range: 0.6-4 cm 2 ); average follow-up time: 5.045 ± 3.47 years (range: 1.15-11.01 years). The average preoperative IKDC score was of 31.63 points (±20.24), the average postoperative IKDC score was of 74.18 points (±20.26). The difference between the post- and preoperative IKDC scores was of 42.55 (±21.05) points, being the minimal score increase of 8.1 points and the maximum score increase of 82.8 points. A statistically significant difference ( p < 0.001) was found between the IKDC scores before and after the surgery. A statistically significant relation ( p = 0.038) was found between the IKDC score increase (the difference between the postoperative and the preoperative scores) and the dimension of the lesion. Conclusions Mosaicplasty with osteochondral autograft transfer, when adequately used, can produce excellent results with great durability and functional impact, low morbidity rates and costs. Expansion of the indication criteria shows promising mid-term and long-term results.

19.
Rev Port Cardiol (Engl Ed) ; 38(5): 349-357, 2019 May.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31221486

RESUMO

INTRODUCTION: Takotsubo syndrome (TTS) is characterized by transient left ventricular (LV) systolic dysfunction. AIM: To characterize a Portuguese population with TTS and to determine their short- and medium-term prognosis. METHODS: We conducted a multicenter study in Portuguese hospitals that included all patients diagnosed with TTS, initially retrospectively and subsequently prospectively. Short- and medium-term clinical complications and mortality were assessed. Independent predictors of in-hospital complications and prognostic factors were determined. RESULTS: A total of 234 patients (210 female, age 68±12 years) were included. During hospitalization, 32.9% of patients had complications: acute heart failure (24.4%), atrial fibrillation (9.0%), ventricular arrhythmias (2.6%), complete atrioventricular block (2.1%), stroke/transient ischemic attack (1.7%), and LV thrombus (1.3%). Chronic kidney disease (CKD) (p=0.02), coronary artery disease (CAD) (p=0.027), lower LV ejection fraction (LVEF) on admission (p=0.003), and dyspnea at presentation (p=0.019) were predictors of in-hospital complications. In-hospital mortality was 2.2%. At the mean follow-up of 33±33 months, all-cause mortality was 4.4%, cardiovascular mortality was 0.9% and TTS recurrence was 4.4%. Prolonged QTc interval on admission was associated with complications in follow-up (p=0.001). CONCLUSION: TTS has a good short- and medium-term prognosis. However, the rate of in-hospital complications is high and should not be overlooked. Dyspnea at presentation, CKD, CAD and lower LVEF on admission were independent predictors of in-hospital complications. Prolonged QTc on admission was associated with complications in follow-up.


Assuntos
Cardiomiopatia de Takotsubo/epidemiologia , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda/fisiologia , Idoso , Causas de Morte/tendências , Eletrocardiografia , Feminino , Seguimentos , Mortalidade Hospitalar/tendências , Humanos , Incidência , Masculino , Portugal/epidemiologia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Cardiomiopatia de Takotsubo/complicações , Cardiomiopatia de Takotsubo/fisiopatologia , Fatores de Tempo , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia
20.
Rev. bras. ortop ; 54(3): 316-321, May-June 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1013716

RESUMO

Abstract Objective Focal osteochondral lesions of the knee are found in two thirds of patients undergoing arthroscopy; their treatment, when isolated and especially in young individuals, remains a debating topic. The present study analyzes the results obtained by the application of the mosaicplasty technique on the treatment of isolated knee femoral condyle osteochondral lesions. Methods Retrospective study of patients submitted tomosaicplasty and to subjective analyseswith pre- and postsurgery International KneeDocumentation Committee (IKDC) scores. Results A total of 13 cases with an average age of 34 years old, with male patients (n = 4; 31%) with an average age of 23 years old (range: 17-31 years old), and female patients (n = 9; 69%) with an average age of 39 years old (range: 16-56 years old); medial versus lateral femoral (n = 11; 85% versus n = 2; 15%); the average size of the lesion was 1.8 cm2 (range: 0.6-4 cm2); average follow-up time: 5.045 ± 3.47 years (range: 1.15-11.01 years). The average preoperative IKDC score was of 31.63 points (± 20.24), the average postoperative IKDC score was of 74.18 points (± 20.26). The difference between the post- and preoperative IKDC scores was of 42.55 (± 21.05) points, being theminimal score increase of 8.1 points andthemaximumscore increaseof82.8 points.Astatistically significant difference (p < 0.001) was found between the IKDC scores before and after the surgery. A statistically significant relation (p = 0.038) was found between the IKDC score increase (the difference between the postoperative and the preoperative scores) and the dimension of the lesion. Conclusions Mosaicplasty with osteochondral autograft transfer, when adequately used, can produce excellent results with great durability and functional impact, low morbidity rates and costs. Expansion of the indication criteria shows promising midterm and long-term results.


Resumo Objetivo Lesões osteocondrais focais do joelho são encontradas em dois terços dos pacientes submetidos a artroscopia; seu tratamento, quando isoladas e, principalmente, em indivíduos jovens, ainda é debatido. O presente estudo analisa os resultados obtidos com a aplicação da técnica de mosaicoplastia no tratamento de lesões osteocondrais isoladas do côndilo femoral do joelho. Métodos Estudo retrospectivo de pacientes submetidos à mosaicoplastia e análise subjetiva com pontuações do International Knee Documentation Committee (IKDC, na sigla em inglês) antes e após a cirurgia. Resultados Um total de 13 casos, com média de idade de 34 anos; pacientes do sexo masculino (n = 4; 31%) apresentaram média de idade de 23 anos (17-31 anos), e pacientes do sexo feminino (n = 9; 69%) apresentaram média de 39 anos; (16-56 anos); femoral medial ou lateral (n = 11, 85% versus n = 2, 15%, respectivamente); o tamanho médio da lesão foi de 1,8 cm2 (0,6-4 cm); o tempo médio de acompanhamento foi de 5,045 ± 3,47 anos (1,15-11,01 anos). A pontuação IKDC média préoperatória foi 31,63 pontos ( ± 20,24), e a pós-operatória foi 74,18 pontos ( ± 20,26). A diferença entre as pontuações IKDC obtidas depois e antes da cirurgia foi de 42,55 ( ± 21,05) pontos, com o aumento mínimo de 8,1 pontos e o aumento máximo de 82,8 pontos. Uma diferença estatística significativa (p < 0,001) foi encontrada entre a pontuação IKDC antes e após a cirurgia. Uma relação estatisticamente significativa (p = 0,038) foi observada entre o aumento da pontuação IKDC (a diferença entre a pontuação pré- e pós-operatória) e as dimensões da lesão. Conclusões A mosaicoplastia com transferência de autoenxerto osteocondral, quando adequadamente usada, pode produzir resultados excelentes com grande durabilidade e impacto funcional, baixas taxas de morbidade e baixos custos. A expansão dos critérios de indicação mostra resultados promissores no médio e longo prazo.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Osteocondrite/cirurgia , Osteocondrite/diagnóstico , Artroscopia , Cartilagem , Cartilagem Articular , Articulação do Joelho
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