Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Apr 2023
ReviewDiagnosis and Management of Cystic Fibrosis Exacerbations.
With the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is acute pulmonary exacerbation (PEx). ⋯ This work has now led to several large-scale clinical trials designed to clarify the treatment paradigm for CF PEx. The primary goal of this review is to provide a summary and update of the pathophysiology, clinical and microbial epidemiology, outcome and treatment of CF PEx, biomarkers for exacerbation, and the impact of highly effective modulator therapy on these events moving forward.
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In cystic fibrosis, a new era has started with the approval and use of highly effective cystic fibrosis transport regulator (CFTR) modulator therapy. As pulmonary function is increasing and exacerbation rate significantly decreases, the current meaning of fungal pulmonary diseases is questioned. During the past couple of decades, several studies have been conducted regarding fungal colonization and infection of the airways in people with cystic fibrosis. ⋯ Whether the percentage will decrease or not will be the task of future evaluations in studies and registry analysis. Using the established definition for different categories of fungal diseases is recommended and should be taken into account if patients are deteriorating without responding to antibiotic treatment. Drug-drug interactions, in particular when using azoles, should be recognized and therapies need to be adjusted accordingly.
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Semin Respir Crit Care Med · Apr 2023
Impact of Digital Technologies on Clinical Care for Adults with Cystic Fibrosis.
The coronavirus disease 2019 pandemic accelerated the implementation of digital technologies, which have now become embedded as essential tools for the management of chronic disease, including cystic fibrosis (CF). Despite subsequent easing of restrictions and because of improved clinical stability resulting from the introduction of highly effective modulator therapy, digital technologies including video and telephone consultations and remote monitoring are likely to remain integral to the future delivery of CF health care. In this article, we explore some of the key developments in digital technologies, barriers to their adoption, and how the CF community is likely to embrace lessons learned from the recent pandemic to help modernize and reshape the future of CF care.