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- Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 PandemicPublication . Herth, Felix JF; Sandhaus, Robert A; Turner, Alice M; Sucena, Maria; Welte, Tobias; Greulich, TimmAlpha 1 Antitrypsin deficiency (AATD) is a hereditary condition characterized by low serum Alpha 1 Antitrypsin (AAT) levels and a predisposition towards early-onset emphysema. Infusion of AAT is the only disease-modifying therapy that can sufficiently raise plasma AAT levels above the putative protective threshold and reduce the decline in lung density loss. Several randomized controlled trials (RCTs) and registry studies support the clinical efficacy of AAT therapy in slowing the progression of AATD-related emphysema and improving survival outcomes. The COVID-19 pandemic has prompted physicians to develop additional strategies for delivering AAT therapy, which are not only more convenient for the patient, but are "COVID-19 friendly", thereby reducing the risk of exposing these vulnerable patients. Intravenous (IV) self-administration of AAT therapy is likely to be beneficial in certain subgroups of patients with AATD and can remove the need for weekly hospital visits, thereby improving independence and well-being. Increasing the awareness of self-administration in AATD through the development of formal guidelines and training programs is required among both physicians and patients and will play an essential role, especially post-COVID-19, in encouraging physicians to consider self-administration for AATD in suitable patients. This review summarizes the benefits of AAT therapy on the clinical endpoints of mortality and quality of life (QoL) and discusses the benefits of self-administration therapy compared with conventional therapy administered by a healthcare professional. In addition, this review highlights the challenges of providing AAT therapy during the COVID-19 pandemic and the potential considerations for its implementation thereafter.
- PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemicPublication . Godinas, Laurent; Iyer, Keerthana; Meszaros, Gergely; Quarck, Rozenn; Escribano-Subias, Pilar; Vonk Noordegraaf, Anton; Jansa, Pavel; D’Alto, Michele; Luknar, Milan; Milutinov Ilic, Senka; Belge, Catharina; Sitbon, Olivier; Reis, Abilio; Rosenkranz, Stephan; Pepke-Zaba, Joanna; Humbert, Marc; Delcroix, MarionBackground: During the COVID-19 pandemic, most of the health care systems suspended their non-urgent activities. This included the cancellation of consultations for patients with rare diseases, such as severe pulmonary hypertension (PH), resulting in potential medication shortage and loss of follow-up. Thus, the aim of the study was to evaluate PH patient health status evolution, access to health care and mental health experience during the early phase of the pandemic. Methods: We conducted an online patient survey, available in 16 languages, between 22/05/2020 and 28/06/2020. The survey included questions corresponding to demographic, COVID-19 and PH related information. Results: 1073 patients (or relatives, 27%) from 52 countries all over the world participated in the survey. Seventy-seven percent (77%) of responders reported a diagnosis of pulmonary arterial hypertension and 15% of chronic thromboembolic PH. The COVID-19 related events were few: only 1% of all responders reported a diagnosis of COVID-19. However, 8% of patients reported health deterioration possibly related to PH, and 4% hospitalization for PH. Besides, 11% of the patients reported difficulties to access their PH expert centre, and 3% interruption of treatment due to shortage of medication. Anxiety or depression was reported by 67% of the participants. Conclusion: Although COVID-19 incidence in PH patients was low, PH related problems occurred frequently as the pandemic progressed, including difficulties to have access to specialized care. The importance of primary health care was emphasized. Further studies are needed to evaluate the long-term consequences of COVID-related PH care disruption.
- Recommendations for interventional pulmonology during COVID-19 outbreak: a consensus statement from the Portuguese Pulmonology SocietyPublication . Guedes, Fernando; Boléo-Tomé, J.P.; Rodrigues, L.V.; Bastos, H.N.; Campainha, S.; de Santis, M.; Mota, L.; Bugalho, A.Coronavirus disease 2019 (COVID-19) is an emerging infectious disease caused by a novel SARS-CoV-2 pathogen. Its capacity for human-to-human transmission through respiratory droplets, coupled with a high-level of population mobility, has resulted in a rapid dissemination worldwide. Healthcare workers have been particularly exposed to the risk of infection and represent a significant proportion of COVID-19 cases in the worst affected regions of Europe. Like other open airway procedures or aerosol-generating procedures, bronchoscopy poses a significant risk of spreading contaminated droplets, and medical workers must adapt the procedures to ensure safety of both patients and staff. Several recommendation documents were published at the beginning of the pandemic, but as the situation evolves, our thoughts should not only focus on the present, but should also reflect on how we are going to deal with the presence of the virus in the community until there is a vaccine or specific treatment available. It is in this sense that this document aims to guide interventional pulmonology throughout this period, providing a set of recommendations on how to perform bronchoscopy or pleural procedures safely and efficiently.