Mihaela Dediu

Background lung ultrasound (LUS) has emerged as a potential radiation-free imaging modality for the evaluation of cystic fibrosis (CF) lung disease. However, its clinical utility, the role of CF-specific LUS scoring systems, and correlations with structural and functional outcomes remain incompletely defined. The objective of this review was to systematically evaluate the role of LUS in cystic fibrosis, with particular focus on CF-specific scoring systems, diagnostic performance, and correlations with imaging and functional disease severity. A systematic search of PubMed and Web of Science was conducted for studies published between January 2016 and January 2026 evaluating LUS in individuals with cystic fibrosis. Prospective and retrospective observational studies assessing LUS findings, scoring systems, or clinical outcomes were included. Screening, data extraction, and risk-of-bias assessment were performed independently by two reviewers in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 recommendations. Due to heterogeneity in study design, ultrasound protocols, and outcomes, results were synthesized qualitatively. Nine studies involving 390 individuals with cystic fibrosis met the inclusion criteria, with most cohorts consisting predominantly of pediatric patients. Four studies developed CF-specific LUS scoring systems, while others applied existing or non-CF-specific scores or descriptive protocols. LUS consistently detected peripheral abnormalities, such as subpleural consolidations, pleural irregularities, and interstitial patterns, and frequently identified lesions not visible on chest radiography. CF-specific LUS scores demonstrated strong correlations with computed tomography (CT) structural severity, spirometry, and lung clearance index (LCI), particularly in patients with moderate-to-advanced disease. Correlations were weaker in cohorts with predominantly mild disease or when non-CF-specific scoring systems were used. LUS showed limited sensitivity for central airway pathology, including bronchiectasis and mucus plugging. LUS is a feasible, radiation-free imaging modality capable of detecting and monitoring peripheral lung disease in cystic fibrosis, particularly when CF-specific scoring systems are applied. While it cannot replace CT for comprehensive structural evaluation, LUS may serve as a valuable complementary tool for longitudinal monitoring. Further standardization and multicentre longitudinal studies are required to define its role in routine CF care.

Background and Objectives: Recognizing the crucial gaps in our understanding of pediatric pneumonia post-SARS-CoV-2 infection, this study aimed to assess the relationship between Pediatric Pneumonia Ultrasound Scores (PedPne) and inflammatory biomarkers. The primary objective of this study is to evaluate the predictive value of PedPne in comparison with inflammatory biomarkers (IL-6 and dNLR) for the development of pneumonia in pediatric patients following SARS-CoV-2 infection. Materials and Methods: This longitudinal observational study collected data from pediatric patients diagnosed with pneumonia after an acute SARS-CoV2 infection. The study focused on analyzing changes in PedPne scores and inflammatory markers such as IL-6 and dNLR from initial admission to follow-up at 7 days. Statistical analysis involved calculating the sensitivity, specificity, and Area Under the Curve (AUC) for each biomarker, alongside regression analysis to determine their hazard ratios for predicting pneumonia development. Results: The analysis identified significant cutoff values for dNLR at 1.88 (sensitivity 77.0%, specificity 85.7%, AUC 0.802, p < 0.001), IL-6 at 6.1 pg/mL (sensitivity 70.3%, specificity 92.9%, AUC 0.869, p < 0.001), and PedPne score at 3.3 (sensitivity 75.7%, specificity 78.6%, AUC 0.794, p < 0.001). Conversely, NLR showed lower diagnostic performance (AUC 0.485, p = 0.327). Regression analysis further highlighted the strong predictive power of these markers, with IL-6 showing a fourfold increase in pneumonia risk (HR = 4.25, CI: 2.07–9.53, p < 0.001), dNLR indicating more than a twofold increase (HR = 2.53, CI: 1.19–6.97, p = 0.006), and PedPne score associated with more than a doubling of the risk (HR = 2.60, CI: 1.33–5.18, p < 0.001). Conclusions: The study conclusively demonstrated that both PedPne ultrasound scores and specific inflammatory biomarkers such as dNLR and IL-6 are significant predictors of pneumonia development in pediatric patients post-COVID-19 infection. These findings advocate for the integration of these biomarkers in routine clinical assessments to enhance the diagnostic accuracy and management of pneumonia in children following SARS-CoV-2 infection.

Background: Optimal nutritional status represents one of the therapeutics goal in patients with cystic fibrosis (pwCF). High-caloric diet with adequate pancreatic enzyme replacement therapy represents daily aims. Studies have shown that the CFTR modulators therapy have a benefic effect on pulmonary function, increasing the spirometry values. But does this new therapy have any effect on improving the nutritional status? Recently papers underlined the benefic effect on patients’ weight, but an optimal nutritional status in pwCF is defined by a BMI percentile above 50. Objective: to find out if CFTR modulators therapy has any effect on improving nutritional status in pwCF. Method: an interventional study was started in May 2022 when the triple therapy was initiated; 12 paediatric patients were enrolled. Each patient had its weight and height measured before the treatment and at 1, 3 and 6 months of treatment. BMI and corresponding percentile were calculated. Results: We found statistically significant differences between mean BMI percentile before and after 3 (34.4 vs 43.8, p= 0.013), respectively 6 months of treatment (34.4 vs 43.4, p= 0.0011). No statistically significant difference was found at 1 month of treatment (p= 0.06). 33.3% of patients had an optimal nutritional status before the new therapy was initiated. Only one patient succeeds to achieve an optimal nutritional status and his BMI percentile increased above percentile 50 at 3 months. He managed to keep this status at 6 months of therapy, when 41.6% of patients had an optimal nutritional status. Conclusions: Patients’ weight increased under CFTR modulator therapy but an optimal nutritional status is hard to be achieved.

In cystic fibrosis (CF), the respiratory disease is the main factor that influences the outcome and the prognosis of patients, bacterial infections being responsible for severe exacerbations. The etiology is often multi-microbial and with resistant strains. The aim of this paper is to present current existing antibiotherapy solutions for CF-associated infections in order to offer a reliable support for individual, targeted, and specific treatment. The inclusion criteria were studies about antibiotherapy in CF pediatric patients. Studies involving adult patients or those with only in vitro results were excluded. The information sources were all articles published until December 2021, in PubMed and ScienceDirect. A total of 74 studies were included, with a total number of 26,979 patients aged between 0–18 years. We approached each pathogen individual, with their specific treatment, comparing treatment solutions proposed by different studies. Preservation of lung function is the main goal of therapy in CF, because once parenchyma is lost, it cannot be recovered. Early personalized intervention and prevention of infection with reputable germs is of paramount importance, even if is an asymmetrical challenge. This research received no external funding.

(1) Background: In cystic fibrosis (CF), the oral glucose tolerance test (OGTT) is recommended from 10 years old annually to screen and diagnose cystic fibrosis-related diabetes (CFRD). Alternative OGTT characteristics (glucose curve shape, time to glucose peak, one-hour glucose value, and three-hour glucose value with the new shape curve) were studied in other populations considered at high risk for diabetes; (2) Methods: The study analyses classical and alternative OGGT characteristics from 44 children (22 CF, 22 obese without CF), mean age: 12.9 ± 2.2 years evaluated in a single-center from Romania. (3) Results: In 59.1% of children with CF, the predominant OGTT pattern was: abnormal glucose metabolism or CFRD, with a monophasic curve shape, a late peak glucose level, and 1 h glucose ≥ 155 mg/dL, showing a very different pattern compared with sex and age-matched obese children. Statistical estimation agreement between the late glucose peak (K = 0.60; p = 0.005), the 1 h glucose ≥ 155 mg/dL during OGTT (K = 0.69, p = 0.001), and the classical method of interpretation was found. (4) Conclusions: Late peak glucose and 1 h glucose level ≥ 155 mg/dL during OGTT can be used for diagnosing the early glucose metabolism alteration in children with CF.

Background: Life expectancy has increased in cystic fibrosis (CF) patients; however, the rate of mortality is still high, and in a majority of cases, the cause of death is due to respiratory deterioration. Vitamin D plays an important role in immunity and infection prophylaxis, as its deficiency is associated with frequent infections. In CF patients, a deficit of liposoluble vitamins is common, despite daily supplementation. The aim of this study is to evaluate the relation between vitamin D status and lung function expressed by lung clearance index (LCI) in patients with CF. We also assessed the relation of factors such as nutritional status, genotype, and associated comorbidities such as Pseudomonas infection, cystic fibrosis-related diabetes (CFRD), and cystic fibrosis liver disease (CFLD) with vitamin D and LCI. Methods: A cross-sectional study was conducted at the National Cystic Fibrosis Center by analyzing patients with CF who presented in our center between November 2017 and November 2019. We enrolled in the study patients diagnosed with CF, who were followed up in our CF center and who were able to perform lung function tests. Patients in exacerbation were excluded. Results: A strong negative correlation was found between vitamin D and LCI (r = −0.69, p = 0.000). A lower vitamin D storage was found in patients with CFLD and CFRD. Higher LCI values were found among patients with chronic Pseudomonas infection, with BMI under the 25th percentile, or with associated CFLD. Conclusion: In CF patients, vitamin D plays an important role, and its deficit correlates with an impaired LCI. Vitamin D deficit is a risk factor in patients with associated comorbidities such as CFLD and CFRD. Chronic infection with Pseudomonas, the presence of impaired nutritional status, and CFLD are associated with a prolonged LCI.

Background: Cystic fibrosis (CF) lung disease determines the outcome of this condition. For lung evaluation processes, computed tomography (CT) is the gold standard, but also causes irradiation. Lately, lung ultrasound (LUS) has proven to be reliable for the diagnosis of consolidations, atelectasis, and/or bronchiectasis. The aim of our study was to evaluate the value of a newly conceived LUS score by comparing it to the modified Bhalla CT score. A further aim was to evaluate the correlation between the score and the lung clearance index (LCI). Methods: Patients with CF were screened by LUS, followed by a CT scan. Spearman’s test was used for correlations. Results: A total of 98 patients with CF were screened, and 57 were included in the study; their mean age was 11.8 ± 5.5 (mean ± SD) years. The mean LUS score was 5.88 ± 5.4 SD. The LUS CF score had a very strong correlation with the CT score of rs = 0.87 (p = 0.000). LUS showed a good sensibility for detecting atelectasis (Se = 83.7%) and consolidations (Se = 94.4%). A lower Se (77.7%) and Sp (9%) were found for cylindrical bronchiectasis. Conclusion: Our study shows that LUS and the lung CF score are parameters that can be used with a complementary role in the diagnosis and monitoring of CF lung disease in children.

Strong>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. </div

Background: Pneumonia is the leading cause of death among children; thus, a correct early diagnosis would be ideal. The imagistic diagnosis still uses chest X-ray (CXR), but lung ultrasound (LUS) proves to be reliable for pneumonia diagnosis. The aim of our study was to evaluate the sensitivity and specificity of LUS compared to CXR in consolidated pneumonia. Methods: Children with clinical suspicion of bacterial pneumonia were screened by LUS for pneumonia, followed by CXR. The agreement relation between LUS and CXR regarding the detection of consolidation was evaluated by Cohen’s kappa test. Results: A total of 128 patients with clinical suspicion of pneumonia were evaluated; 74 of them were confirmed by imagery and biological inflammatory markers. The highest frequency of pneumonia was in the 0–3 years age group (37.83%). Statistical estimation of the agreement between LUS and CXR in detection of the consolidation found an almost perfect agreement, with a Cohen’s kappa coefficient of K = 0.89 ± 0.04 SD, p = 0.000. Sensitivity of LUS was superior to CXR in detection of consolidations. Conclusion: Lung ultrasound is a reliable method for the detection of pneumonia consolidation in hospitalized children, with sensitivity and specificity superior to CXR. LUS should be used for rapid and safe evaluation of child pneumonia.

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 (FEV₁), 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 FEV₁ was 1.52% lower with every year rise of age. PI and female sex increased the risk of impaired lung function (FEV₁ < 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.