Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study

Bain, Robert; Cosgriff, Rebecca; Zampoli, Marco; Elbert, Alexander; Burgel, P.-R.; Carr, Siobhán; Castaños, Claudio; Colombo, Carla; Corvol, Harriet; Faro, Albert; Goss, Christopher H.; Gutierrez, Hector; Jung, Anna; Kashirskaya, N.; Marshall, Bruce C.; Melo, Joel; Mondéjar-López, Pedro; Monestrol, Isabelle de; Naehrlich, L.; Padoan, Rita; Pastor-Vivero, María Dolores; Rizvi, Samar; Salvatore, Marco; Filho, Luiz Vicente Ribeiro Ferreira da Silva; Brownlee, K.; Haq, Iram; Brodlie, Malcolm

doi:10.1016/j.jcf.2020.11.021

Cited by 71 publications

(144 citation statements)

References 37 publications

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“…Therefore, the SARS-CoV-2 pandemic caused considerable anxiety in the CF community [4] . Early case series of pwCF in various regions who tested positive for SARS-CoV-2 suggest that pwCF have better outcomes than expected [5] , [6] , [7] , [8] .…”

Section: Introductionmentioning

confidence: 99%

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Naehrlich

Orenti

Dunlevy³

et al. 2021

Journal of Cystic Fibrosis

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Background Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF). Methods We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection. Results Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/1000 pwCF. Incidence was higher in lung-transplanted patients (n=23) versus non-transplanted patients (n=107) (8.43 versus 2.36 cases/1000). Incidence was higher in pwCF versus the age-matched general population in the age groups <15, 15-24, and 25-49 years (p<0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p=0.133). Conclusions SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination.

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Section: Introductionmentioning

confidence: 99%

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Naehrlich

Orenti

Dunlevy³

et al. 2021

Journal of Cystic Fibrosis

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“…Therefore, patients having pre-existing lung problems, such as chronic obstructive pulmonary disease (COPD), severe asthma and CF lung disease, should be at high risk of developing severe symptoms of COVID-19 [44]. Surprisingly, the preliminary data collected by the European Cystic Fibrosis Society on the response of CF patients to COVID-19 suggest that the course of the disease may be milder than expected [45]. We have recently reported our hypothesis that low concentrations of IL-6 present in the inflamed airway tract of CF patients might not only be the hallmark of CF lung inflammation, but also an indicator of the inhibition of the cytokine storm and the predictor of diseases severity in the course of COVID-19 superinfection [46].…”

Section: Sputum Biomarkers In Cystic Fibrosismentioning

confidence: 99%

Chronic bacterial pulmonary infections in advanced cystic fibrosis differently affect the level of sputum neutrophil elastase, IL-8 and IL-6

Majka

Mazurek²,

Strus

et al. 2021

Clinical and Experimental Immunology

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“…The authors concluded that children with CF experience a similar COVID-19 disease course compared to children without CF. 14 Likewise, the expert panel from the European Respiratory Society (ERS) examined the response of children with chronic respiratory conditions to COVID-19 by collating data from 174 ERS centres. Fourteen children with CF were reported to have COVID-19.…”

Section: Cystic Fibrosis and Severe Acute Respiratory Syndrome Coronavirus 2: Epidemiologymentioning

confidence: 99%

SARS-CoV-2 and Cystic Fibrosis: Expectations Versus Reality, a Literature Review

Bantounou

Plascevic

2021

EMJ Respir

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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), responsible for the COVID-19 pandemic, is expected to cause severe illness in people with cystic fibrosis (CF). The infection prevalence and clinical outcomes of this patient cohort to SARS-CoV-2 were explored, alongside contributing factors to the observed response. Search terms were entered into Medline/PubMed and Embase databases, with relevant published papers written in English chosen. The COVID-19 trajectory in people with CF (including children) was similar to the general population. Specifically, in Veneto, Italy, the infection rate of people with CF was nearly half compared to the general population (0.19% versus 0.40%, respectively). Similarly, in Spain, the cumulative incidence of COVID-19 was lower compared to the general population: 32/10,000 and 49/10,000 respectively. Likewise, in Belgium 2.7% of patients with CF had SARS-CoV-2 antibodies compared with 4.3% of the general population. Moreover, in Europe, fewer CF–COVID-19 cases and deaths were reported compared to the general population (1.1%, 0.9%; and 3.2%, 2.3%, respectively). Overall, worse outcomes in CF were associated with poorer lung function and post-transplant status. The encouraging response of people with CF to COVID-19 is hypothesised as due to higher levels of anti-inflammatory angiotensin-1-7 and lower levels of pro-inflammatory IL-6 and protease transmembrane serine protease-2, utilised by SARS-CoV-2 to penetrate cells. Additionally, the use of CF medication, chiefly Dornase alfa and CF transmembrane conductance regulator modulators as well as CF cohort characteristics, predominantly younger age, and early isolation might have mitigated COVID-19 severity. Thus, people with CF do not appear to have a higher COVID-19 infection prevalence or worse clinical outcomes compared to the general population.

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Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study

Cited by 71 publications

References 37 publications

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

Chronic bacterial pulmonary infections in advanced cystic fibrosis differently affect the level of sputum neutrophil elastase, IL-8 and IL-6

SARS-CoV-2 and Cystic Fibrosis: Expectations Versus Reality, a Literature Review

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