The global impact of SARS-CoV-2 in 181 people with cystic fibrosis

McClenaghan, Elliot; Cosgriff, Rebecca; Brownlee, K.; Ahern, Susannah; Burgel, P.-R.; Byrnes, Catherine A.; Colombo, Carla; Corvol, Harriet; Cheng, Stephanie; Daneau, Géraldine; Elbert, Alexander; Faro, Albert; Goss, Christopher H.; Gulmans, Vincent; Gutierrez, Hector; Monestrol, Isabelle de; Jung, Anna; Justus, Lutz Nährlich; Kashirskaya, N.; Marshall, Bruce C.; McKone, Edward F.; Middleton, Peter G.; Mondéjar-López, Pedro; Pastor-Vivero, María Dolores; Padoan, Rita; Rizvi, Samar; Ruseckaite, Rasa; Salvatore, Marco; Stephenson, Anne L.; Filho, Luiz Vicente R. da Silva; Melo, Joel; Zampoli, Marco; Carr, Siobhán

doi:10.1016/j.jcf.2020.10.003

Cited by 80 publications

(119 citation statements)

References 6 publications

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“…Very recently, the international Cystic Fibrosis Registry Global Harmonization Group published in this Journal interesting data on the clinical characteristics and outcomes of SARS-Cov-2 infection in CF patients. [2] …”

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confidence: 99%

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First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry

Padoan

Carnovale

Salvatore

et al. 2021

Journal of Cystic Fibrosis

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confidence: 99%

“…These data are in accordance with those reported by the clinical studies of the global project. [2] .…”

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confidence: 99%

First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry

Padoan

Carnovale

Salvatore

et al. 2021

Journal of Cystic Fibrosis

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“…In particular, an early multinational report on 40 CF cases highlighted good recovery from SARS-CoV-2 in the heterogeneous 40-subject CF cohort, with a total absence of mortality that may derive from the relatively low incidence of the novel coronavirus amongst the CF population [ 155 ]. A subsequent report involving a larger international cohort (181 people with CF) confirmed that SARS-CoV-2 infection for most people with CF may be less severe than originally feared, although it can result in serious consequences for some CF patients (with older age, CF-related diabetes, lower lung function, and recipients of an organ transplant) [ 156 ]. Moreover, it has recently emerged that a one-month-old infant with CF and SARS-CoV-2 positivity did not display any COVID-19 symptoms, suggesting that the low pathogenicity observed in healthy children applies to young CF patients as well [ 157 ].…”

Section: Potential Impact Of Covid-19 On Inflammation In Cf Patienmentioning

confidence: 99%

Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches

Ghigo

Prono

Riccardi

et al. 2021

IJMS

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Cystic fibrosis (CF) is an inherited disorder caused by mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP-gated chloride channel expressed on the apical surface of airway epithelial cells. CFTR absence/dysfunction results in defective ion transport and subsequent airway surface liquid dehydration that severely compromise the airway microenvironment. Noxious agents and pathogens are entrapped inside the abnormally thick mucus layer and establish a highly inflammatory environment, ultimately leading to lung damage. Since chronic airway inflammation plays a crucial role in CF pathophysiology, several studies have investigated the mechanisms responsible for the altered inflammatory/immune response that, in turn, exacerbates the epithelial dysfunction and infection susceptibility in CF patients. In this review, we address the evidence for a critical role of dysfunctional inflammation in lung damage in CF and discuss current therapeutic approaches targeting this condition, as well as potential new treatments that have been developed recently. Traditional therapeutic strategies have shown several limitations and limited clinical benefits. Therefore, many efforts have been made to develop alternative treatments and novel therapeutic approaches, and recent findings have identified new molecules as potential anti-inflammatory agents that may exert beneficial effects in CF patients. Furthermore, the potential anti-inflammatory properties of CFTR modulators, a class of drugs that directly target the molecular defect of CF, also will be critically reviewed. Finally, we also will discuss the possible impact of SARS-CoV-2 infection on CF patients, with a major focus on the consequences that the viral infection could have on the persistent inflammation in these patients.

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“…As we transition into the winter months marked with a significant rise of SARS-CoV-2 in many parts of the country, it remains imperative that we continue collecting survey data. This effort paired with others across the globe [7] with allow us to monitor and report out on the impact of SARS-CoV-2 on clinical trials networks as well as the health and well-being of people with CF.…”

Section: Next Stepsmentioning

confidence: 99%

The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network

Pearson

Mayer-Hamblett²,

Goss

et al. 2021

Journal of Cystic Fibrosis

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The Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV-2) global pandemic significantly impacted CF clinical research within the Cystic Fibrosis Foundation Therapeutics Development Network (CFF TDN). A Research Electronic Data Capture (REDCap) survey was developed and sent to network sites to monitor and understand the impact on research teams, ongoing and anticipated clinical research, and specific clinical and research procedures. Key findings indicated an early impact on participant enrollment, research team stability, and procedures such as spirometry and sputum induction. These trends steadily improved over the months as research activities began to recover across the TDN. While SARS-CoV-2 created a significant challenge it also highlights new opportunities to expand CF research with greater focus on data collection outside of research centers and increased access for remote participation.

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The global impact of SARS-CoV-2 in 181 people with cystic fibrosis

Cited by 80 publications

References 6 publications

First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry

First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry

Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches

The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network

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