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.

BackgroundSARS-Co-V-2 infection in people with CF (pwCF) can lead to severe outcomes.MethodsIn this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis.ResultsUp to 31 December 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection. Incidence was 17.2 per 1000 pwCF (95% CI: 16.0–18.4). Median age was 24 years, 48.4% were male and 9.4% had lung transplants. SARS-CoV-2 incidence was higher in lung-transplanted (28.6 [95% CI: 22.7–35.5]) versus non-lung transplanted pwCF (16.6 [95% CI: 15.4–17.8]) (p=<0.001).SARS-CoV-2 infection caused symptomatic illness in 75.7%. Factors associated with symptomatic SARS-CoV-2 infection were age >40 years, at least one F508del mutation, and pancreatic insufficiency.Overall, 23.7% were admitted to hospital, 2.5% to intensive care. Regretfully 11 pwCF (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2–6-fold more frequent in lung-transplanted versus non-lung transplanted pwCF.Factors associated with hospitalisation and oxygen therapy were lung transplantation, CF-related diabetes (CFRD), moderate or severe lung disease and azithromycin use (often considered a surrogate marker for Pseudomonas aeruginosa infection and poorer lung function).ConclusionSARS-CoV-2 infection yielded high morbidity and hospitalisation in pwCF. PwCF with forced expiratory volume in one second (FEV1) <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.

No abstract

We consider that the frequency of F508del in CF patients from Romania is higher than in previous reports, reaching 56.3%, probably owing to more rigorous selection of patients for genetic testing, allowing improved calculation of mutation frequencies.

Genetic analysis of 32 children with cystic fibrosis from Romania showed a 25% incidence of DeltaF508 mutation and a 64.5% incidence of unknown mutations, 5 other known mutations and 1 new mutation 1,717-2(A > G).

CF carriers of a severe genotype which associates pancreatic insufficiency and CF liver disease, are more likely predisposed to low bone mineral density. Further studies should discover other significant influences in order to prevent the development of CF bone disease and an improved quality of life in cystic fibrosis children.

Purpose The aim of this cross-sectional study was to identify the major factors influencing pulmonary function in CF patients from western side of Romania. Patients and Methods The study enrolled 51 patients with CF between the ages of 6 and 27.8 years who were monitored at regular visits to the National Cystic Fibrosis Centre and Pius Branzeu County Hospital in Timisoara, Romania, over a period of 2 years. The relationships between lung function, as measured by forced expiratory volume in 1 s (FEV 1 ), with patient age, sex, body mass index (BMI), pancreatic insufficiency (PI), microbial infection, CF-related diabetes (CFRD), CF-associated liver disease (CFLD), and physical activity <30 min/day, were evaluated by multivariate regression analysis. Results The results showed that FEV1 was 0.32% higher for each increase in percentile of BMI (95% confidence interval: 0.034–0.619). In addition, mean FEV 1 was 1.52% lower with every year rise of age. PI and female sex increased the risk of impaired lung function (FEV 1 <60%). The factors most closely correlated with pulmonary function in pediatric CF patients were current age (negative) and BMI (positive). Conclusion The findings of this study, in combination with what is known from other studies in CF, suggest that a better nutritional status and infection prophylaxis should be part of a comprehensive clinical management strategy for pediatric CF in Romania.

Introduction Pneumonia is the principal cause of death among children worldwide. Lung ultrasound (LUS) is a reliable tool for the diagnosis and assessment of community‐acquired pneumonia in children. Furthermore, objective parameters, including the pneumonia LUS score, might be useful for pneumonia monitoring. Thus, our aim was to present a newly developed LUS score for pediatric pneumonia (PedPne) and evaluate its relationship with commonly assessed inflammatory markers. Methods Children referred to the Pediatric Pneumology Clinic between September 2017 and February 2018 with suspected pneumonia were screened for eligibility for inclusion in the study and informed consent was obtained. In addition to clinical assessment, LUS was performed during consultation, and inflammatory biomarkers, including C‐reactive protein level, erythrocyte sedimentation rate (ESR), and leukocyte count, were measured in all inpatients. An LUS score for pneumonia and pleurisy in children (pediatric pneumonia lung ultrasound score [PedPne LUS]) was subsequently developed. Chest radiography (CXR) was also performed according to local guidelines for pneumonia diagnosis. Spearman's correlation test was used to evaluate the correlation between the PedPne score and inflammatory markers. Results A total of 217 patients were screened, of which 64 patients diagnosed with consolidated pneumonia were included in this study. The median PedPne LUS score of the included patients was 8.02, which was consistent with the consolidations detected on LUS and confirmed by CXR. A very strong positive correlation was found between the LUS PedPne score and C‐reactive protein and ESR, and a good correlation was found with the leukocyte count. Conclusion The LUS pneumonia score is a reliable parameter for the evaluation of pneumonia, and shows a strong correlation with inflammatory biomarkers. The PedPne LUS score is a potential noninvasive surrogate parameter of inflammation in pediatric pneumonia.