Pneumologia
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- O Alívio dos Sintomas do Doente com Doença Respiratória em Fim de VidaPublication . Gonçalves, AOs cuidados que se prestam ao doente com doença respiratória crónica em fim de vida, representam um desafio para os profissionais de saúde, especialmente para os enfermeiros que os acompanham nesta etapa. Nos últimos anos assistiu-se a um aumento de doenças crónicas que afectam o aparelho respiratório. Estas levam muitas vezes a uma diminuição da qualidade de vida e aumento da dependência por parte dos doentes. No que diz respeito às doenças respiratórias raramente existe um acontecimento que indique o fim de vida, uma vez que se trata de um processo gradual de deterioração, ao longo de muitos anos. Assim, os enfermeiros devem estar despertos para pequenas alterações no estado físico, psicossocial e na sintomatologia do doente que indiquem o início deste processo. Os sintomas respiratórios constituem um importante conjunto de problemas que surgem no fim da vida de pessoas vítimas de doença do foro respiratório, podendo no entanto estar presentes noutras situações. O controlo destes sintomas depende em grande parte das medidas implementadas pelas equipas de saúde no alívio dos mesmos.
- Alpha-1 Antitrypsin Deficiency: Principles of CarePublication . Rodrigues, J; Mineiro, A; Reis, A; Ventura, D; Fernandez-Llimos, F; Costa, F; Gomes, J; Silva, JM; Lopes, P; Robalo Cordeiro, CAlpha-1 antitrypsin deficiency is an autosomal co-dominant inherited disorder that results in decreased circulating levels of alpha-1 antitrypsin (also known as alpha-1 proteinase inhibitor) and predisposes affected individuals to early onset lung and liver disease. There is currently no cure for alpha-1 antitrypsin deficiency. However, appropriate treatment and a high standard of clinical care can prevent patients from being seriously affected and having to undergo major medical interventions, such as organ transplantation. Beyond managing the symptoms associated with alpha-1 antitrypsin deficiency, alpha-1 proteinase inhibitor therapy is the only treatment for the condition's underlying cause. Early diagnosis is important to ensure efficient therapeutic strategies and to minimize further deterioration of lung function. alpha-1 antitrypsin deficiency is under diagnosed globally, partly because the disease has no unique presenting symptoms. This document was prepared by a Portuguese multidisciplinary group and it aims to set out comprehensive principles of care for Alpha-1 antitrypsin deficiency. These include the importance of registries, the need for clinical research, the need for consistent recommendations (regarding diagnosis, treatment and monitoring), the role of reference centres, the requirement for sustained access to treatment, diagnostic and support services, and the role of patient organizations.
- An Atypical Presentation of Thrombotic Microangiopathy After Lung Transplant: a Case ReportPublication . Menezes, MM; Aires, I; Semedo, L; Calado, J; Ribeiro, F; Nolasco, FThrombotic microangiopathy (TMA) is a pathologic condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury due to microvascular endothelial lesions and thrombosis. It occurs in a variety of diseases and, unless recognized and treated, leads to severe morbidity and mortality. We present the case of a 48-year-old woman who underwent lung transplantation, initially under tacrolimus, mycophenolate mofetil (MMF), and prednisolone. Several complications emerged in the following months, including abdominal aortic and left renal artery thrombosis and cutaneous infections, although her renal function remained normal. Six months after transplant, her renal function began to deteriorate, which was assumed to be due to elevated tacrolimus levels and doses were adjusted. Due to leukopenia, MMF was changed to everolimus. One year after, she was admitted with fatigue, anemia, and renal dysfunction. Complementary exams revealed only iron deficiency, leukopenia, normal platelets, and elevated lactate dehydrogenase; her renal ultrasound was normal. A renal biopsy was performed and thrombotic microangiopathy was subsequently identified as the main cause of the renal dysfunction. Tacrolimus was therefore discontinued and MMF restarted with slow improvement of renal function. Only when everolimus was stopped did the patient's renal function show incremental improvement. TMA may be a serious complication after lung transplantation and the risk is higher when a combination of tacrolimus and everolimus is used. Renal biopsy findings are essential to confirm the final diagnosis of TMA, allowing for a change in immunosuppression to prevent permanent and severe renal damage.
- Assimetria Pulmonar na Telerradiografia do TóraxPublication . Oliveira, R; Martins, JD; Marques, H; Oliveira Santos; Freitas, I; Pinto, MFA ausência unilateral de uma artéria pulmonar é uma anomalia congénita rara. Os autores descrevem o caso de um rapaz de dois anos, sem antecedentes patológicos prévios e que é referenciado para avaliação após a detecção na telerradiografia de tórax de assimetria dos campos pulmonares com desvio do mediastino para a direita. A tomografia axial computorizada e a cintigrafia de perfusão pulmonar entretanto efectuadas, indicavam para a ausência da artéria pulmonar direita que foi comprovada no cateterismo cardíaco e em ressonância magnética. Esta é uma patologia relevante pois o seu diagnóstico precoce e a sua correcção atempada podem evitar morbilidades no futuro. Dada a idade e o facto de o doente estar de momento assintomático, optou-se por uma atitude conservadora e vigilância em ambulatório.
- Asthma Management With Breath-Triggered Inhalers: Innovation Through DesignPublication . Morais-Almeida, M; Pité, H; Cardoso, J; Costa, R; Robalo Cordeiro, C; Silva, E; Todo-Bom, A; Vicente, C; Marques, JABackground: Asthma affects the lives of hundred million people around the World. Despite notable progresses in disease management, asthma control remains largely insufficient worldwide, influencing patients' wellbeing and quality of life. Poor patient handling of inhaling devices has been identified as a major persistent problem that significantly reduces inhaled drugs' efficacy and is associated with poor adherence to treatment, impairing clinical results such as asthma control and increasing disease-related costs. We herein review key research and development (R&D) innovation in inhaler devices, highlighting major real-world critical errors in the handling and inhalation technique with current devices and considering potential solutions. Furthermore, we discuss current evidence regarding breath-triggered inhalers (BTI). Main body: The two most common significant problems with inhalers are coordinating actuation and inhalation with pressurized metered-dose inhalers (pMDIs), and the need to inhale forcibly with a dry powder inhaler. BTI R&D plans were designed to overcome these problems. Its newest device k-haler® has several other important features, generating a less forceful aerosol plume than previous pMDIs, with efficient drug delivery and lung deposition, even in patients with low inspiratory flow. The local and systemic bioavailability of fluticasone propionate and formoterol (FP/FORM) administered via k-haler® has been shown to be therapeutically equivalent when administered via the previous FP/FORM pMDI. This device requires very few steps and has been considered easy to use (even at first attempt) and preferred by the patients in a randomized crossover study. In our country, FP/FORM k-haler is available without additional costs compared to FP/FORM pMDI. All devices continue to require education and regular checking of the correct inhalation technique. Conclusion: BTI R&D can bring advantage over current available inhalers, avoiding the two most common identified critical errors in inhalation technique. K-haler® BTI is currently available, without an increased cost, and approved for adolescents and adults with asthma in whom treatment with inhaled combined therapy with long-acting beta2-agonists and corticosteroids is indicated. Its attractive and practical design to facilitate its use has been awarded. K-haler® represents added value through innovation to fulfill actual asthma patient needs, thus with potential relevant impact in asthma management and effective control.
- Asthma-Chronic Obstructive Pulmonary Disease Overlap Syndrome - Literature Review and Contributions Towards a Portuguese ConsensusPublication . Araújo, D; Padrão, E; Morais-Almeida, M; Cardoso, J; Pavão, F; Leite, RB; Caldas, AC; Marques, AINTRODUCTION: Phenotypic overlap between the two main chronic airway pulmonary diseases, asthma and chronic obstructive pulmonary disease (COPD), has been the subject of debate for decades, and recently the nomenclature of asthma-COPD overlap syndrome (ACOS) was adopted for this condition. The definition of this entity in the literature is, however, very heterogeneous, it is therefore important to define how it applies to Portugal. METHODS: A literature review of ACOS was made in a first phase resulting in the drawing up of a document that was later submitted for discussion among a panel of chronic lung diseases experts, resulting in reflexions about diagnosis, treatment and clinical guidance for ACOS patients. RESULTS: There was a consensus among the experts that the diagnosis of ACOS should be considered in the concomitant presence of: clinical manifestations characteristic of both asthma and COPD, persistent airway obstruction (post-bronchodilator FEV1/FVC<0.7), positive response to bronchodilator test (increase in FEV1 of ≥200mL and ≥12% from baseline) and current or past history of smoking or biomass exposure. In reaching diagnosis, the presence of peripheral eosinophilia (>300eosinophils/μL or >5% of leukocytes) and previous history of atopy should also be considered. The recommended first line pharmacological treatment in these patients is the ICS/LABA association; if symptomatic control is not achieved or in case of clinical severity, triple therapy with ICS/LABA/LAMA may be used. An effective control of the exposure to risk factors, vaccination, respiratory rehabilitation and treatment of comorbidities is also important. CONCLUSIONS: The creation of initial guidelines on ACOS, which can be applied in the Portuguese context, has an important role in the generation of a broad nationwide consensus. This will give, in the near future, a far better clinical, functional and epidemiological characterization of ACOS patients, with the ultimate goal of achieving better therapeutic guidance.
- Asthma-COPD Overlap: A Portuguese SurveyPublication . Padrão, E; Araújo, D; Todo Bom, A; Robalo Cordeiro, C; Correia de Sousa, J; Cardoso, J; Morais-Almeida, M; Costa, R; Pavão, F; Leite, RB; Marques, AINTRODUCTION: The overlap between asthma and chronic obstructive pulmonary disease (COPD) (ACO) has been discussed for many years but clinical recommendations for this entity have been diverse. This study is intended to reach a consensus on diagnosis, treatment and patient orientation for ACO, within the Portuguese medical community. METHODS: This study was conducted by a multidisciplinary panel of experts from three distinct medical specialties (Pulmonology, Family Medicine and Immunoallergology). This panel selected a total of 190 clinicians, based on their expertise in obstructive airway diseases, to participate in a Delphi structured survey with three rounds of questionnaires. These results were ultimately discussed, in a meeting with the panel of experts and some of the study participants, and consensus was reached in terms of classification criteria, treatment and orientation of ACO patients. RESULTS: The majority of clinicians (87.2%) considered relevant the definition of an overlap entity between asthma and COPD. A consensus was achieved on the diagnosis of ACO - presence of simultaneous clinical characteristics of asthma and COPD together with a fixed airflow obstruction (FEV1/FVC<0.7) associated with 2 major criteria (previous history of asthma; presence of a previous history of smoking exposure and/or exposure to biomass combustion; positive bronchodilation test (increase in FEV1 of at least 200mL and 12%) on more than 1 occasion) plus 1 minor criteria (history of atopy; age ≥40 years; peripheral eosinophilia (>300eosinophils/μL or >5% of leukocytes); elevation of specific IgEs or positive skin tests for common allergens). A combination of inhaled corticosteroid (ICS) with long-acting beta2-agonist (LABA) or long-acting muscarinic antagonist (LAMA) was considered as first line pharmacological treatment. Triple therapy with ICS plus LABA and LAMA should be used in more severe or symptomatic cases. Non-pharmacological treatment, similar to what is recommended for asthma and COPD, was also considered highly important. A hospital referral of ACO patients should be made in symptomatic or severe cases or when there is a lack of diagnostic resources. CONCLUSIONS: This study highlights the relevance of defining ACO, within the Portuguese medical community, and establishes diagnostic criteria that are important for future interventional studies. Recommendations on treatment and patient's orientation were also achieved.
- Broncho-Esophageal Fistula: When Surgery and Endoscopy Fail, Consider Physiological Lung ExclusionPublication . Maia, D; Tronchetti, J; D'Journo, X; Dutau, HWe discuss the case of an esophageal cancer patient treated by chemo and radiotherapy complicated by an esophageal stenosis and an iatrogenic broncho-esophageal fistula. This latter was managed with multiple palliative stenting procedures and colonic surgical bypass. Despite a long disease free survival but decreased quality of life and frailty, we came to the proposal of an extremely unusual form of treatment - physiological lung exclusion, with clinical benefit and so far without any drawbacks related to the procedure.
- Chronic Obstructive Pulmonary Disease Guidelines in Europe: a Look Into the FuturePublication . Miravitlles, M; Roche, N; Cardoso, J; Halpin, D; Aisanov, Z; Kankaanranta, H; Kobližek, V; Śliwiński, P; Bjermer, L; Tamm, M; Blasi, F; Vogelmeier, CFClinical practice guidelines are ubiquitous and are developed to provide recommendations for the management of many diseases, including chronic obstructive pulmonary disease. The development of these guidelines is burdensome, demanding a significant investment of time and money. In Europe, the majority of countries develop their own national guidelines, despite the potential for overlap or duplication of effort. A concerted effort and consolidation of resources between countries may alleviate the resource-intensity of maintaining individual national guidelines. Despite significant resource investment into the development and maintenance of clinical practice guidelines, their implementation is suboptimal. Effective strategies of guideline dissemination must be given more consideration, to ensure adequate implementation and improved patient care management in the future.
- Clinical and Molecular Markers in COPDPublication . Gonçalves, I; Guimarães, MJ; van Zeller, M; Menezes, F; Moita, J; Simão, PChronic obstructive pulmonary disease (COPD) is a complex and heterogeneous disease, and there is a clinical need for validated markers and biomarkers that can contribute to the assessment of patients, risk prediction, treatment guidance, and assessment of response. Although according to the 2018 GOLD guidelines clinically useful biomarkers for COPD patients in stable condition have yet to be identified, several clinical markers and biomarkers have been proposed for COPD. These include isolated clinical markers, such as symptoms and Health Status assessment, exercise tests, function tests and imaging, and also composite scores and molecular markers. However, and despite strong efforts to identify useful markers in an attempt to improve prognostic and therapeutic approaches, results have not been consistent and expectations of relying on these markers in near future are faint. Current approaches to COPD have shifted from treating the disease to treating the individual patient. There is a clear need to identify treatable traits, focusing more on the patient and not on the disease, in order to implement an increasingly personalized treatment of COPD in the clinic, leading to true precision medicine. There is a need to identify combinations of clinical markers and biomarkers, genetic markers, and phenotypes that can guide the personalized therapy of COPD patients. This critical review will therefore focus not only on currently established markers and biomarkers in COPD but also on possible future approaches toward precision medicine.