Acute Pulmonary Exacerbations in Cystic Fibrosis

Abstract

With the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator 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 an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the disease course, prognosis, and complications. This work has now led to a number of large scale clinical trials with the goal of improving the treatment paradigm for CF pulmonary exacerbation. The primary goal of this review is to provide a summary of the pathophysiology, the clinical epidemiology, microbial epidemiology, outcome and the treatment of CF pulmonary exacerbation.

Impact of Newborn Screening for Cystic Fibrosis on Clinical Outcomes of Pediatric Patients: 10 Years’ Experience in Lodz Voivodship.

“…[17] The occurrence of respiratory exacerbations are associated with an accelerated decline in lung function and reduced quality of life and survival. [15][16][17] On the other hand mean values of FEV1 in the analysed patients, presented stable results during at least 3 years of observation; there was no difference between Groups. This may be due to the age -below 8 -too early to identify signi cant differences in FEV1 secondary to clinical outcome (such as pulmonary exacerbations, bacterial colonisations), which likely takes longer to cause impairment of lung function that is identi able with spirometry.…”

“…It is very bene cial observation due to reports which emphasise that the repeated mildto-moderate pulmonary exacerbations especially in the rst years of life result in airway remodelling, while more severe hospitalised episodes could increase structural airway injury risk. [13][14][15][16] Data from Australia showed improved survival in future in patients with lower number of hospitalisations in the rst 3 years of life. [17] The occurrence of respiratory exacerbations are associated with an accelerated decline in lung function and reduced quality of life and survival.…”

“…This may be due to the age -below 8 -too early to identify signi cant differences in FEV1 secondary to clinical outcome (such as pulmonary exacerbations, bacterial colonisations), which likely takes longer to cause impairment of lung function that is identi able with spirometry. [16] Children with CF are highly susceptible to chronic respiratory tract colonization and subsequent recurrent infection. It is know that once chronic PA infection is established, the risk of mortality and morbidity increases.…”

COVID-19 and Cystic Fibrosis: Diagnostic Difficulties or Incorrect Methods?

“…When comparing the tomography of September 2019 with the tomography of April 2020 (Fig. 2), we found that both present chronic lesions typical of cystic fibrosis and patches of ground glass patches of subpleural distribution in the basal lobes, which corresponds to the process chronic inflammatory 2,9,15 .…”

“…It is recognized that viral infections in patients with cystic fibrosis are a factor of poor prognosis and deterioration of lung function and increased frequency of PE and its severity 2,7,9,10 . The symptoms of a SARS-CoV-2 infection differ from the symptoms of PE that most patients with cystic fibrosis know how to recognize 5,7 .…”