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.
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.
(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.
Objectives: Congenital heart defects (CHDs) are among the most frequent congenital defects, and they significantly burden the healthcare system due to their high mortality rate and high cost of care for survivors. We aimed to highlight the incidence of CHDs in a tertiary center in Western Romania. Methods: A retrospective study was carried out between 2018 and 2022 at the “Pius Brinzeu” Emergency County Hospital Timisoara. Relevant information about the mothers and the newborns were collected and statistically analyzed. Results: The incidence of CHDs from 2018 to 2022 in our center was 5.3%. Eleven types of malformations were diagnosed postnatally in 541 newborns, with 28.8% of cases having more than one type of CHD. The antenatal detection rate was 28%, with the highest rates for tetralogy of Fallot, hypoplastic left heart syndrome, or significant ventricular septal defects and the lowest for pulmonary stenosis. The lower antenatal detection rate was influenced mainly by incomplete or absent prenatal care. Conclusions: The incidence of CHDs is clearly dependent of a multifactorial approach, and the results highlight this. With an incidence almost 50% lower than reported within the literature and a low rate of prenatal detections, CHDs could be a more of a burden to endure regarding medical treatment. Improvements in patients’ education, prenatal care, and screening programs could improve diagnosis, decrease mortality, and optimize postnatal care.
Background/Objectives: Highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy has changed cystic fibrosis (CF) care, but Romanian patient-reported health-related quality of life (HRQoL) data remain limited. This national cross-sectional mixed-methods survey aimed to describe patient- and parent-reported HRQoL in Romanian people with CF (pwCF) aged ≥6 years using age-appropriate Cystic Fibrosis Questionnaire-Revised (CFQ-R) versions, with the respiratory domain as the primary outcome. Methods: The online survey was conducted between February and April 2026. Respondents provided demographic and clinical data, including genotype/F508del status, CFTR modulator status, percent predicted forced expiratory volume in 1 s (ppFEV1) category, weight, height, and IV antibiotic-treated pulmonary exacerbations in the previous year. CFQ-R scores were summarized descriptively, and exploratory unadjusted associations were assessed using Mann–Whitney U tests and Spearman correlations. Open-ended responses were analyzed descriptively. Results: Of 67 responses, 61 were included: 43 participants aged 6–13 years and 18 aged ≥14 years. Most respondents were currently receiving CFTR modulators (52/61, 85.2%), predominantly elexacaftor/tezacaftor/ivacaftor. Mean CFQ-R Respiratory scores were 74.5 ± 23.1 in parent-proxy questionnaires for children aged 6–13 years, 83.3 ± 11.8 in self-reported questionnaires for children aged 12–13 years, and 69.8 ± 27.4 among respondents aged ≥14 years. Respiratory scores were higher among currently treated respondents and those with at least one F508del variant, and lower among those reporting IV antibiotic-treated exacerbations. Treatment burden remained among the lower-scoring domains. Qualitative responses described perceived respiratory, nutritional, and functional improvements, alongside residual treatment burden, psychosocial challenges, and access-related expectations. Conclusions: This national Romanian mixed-methods survey provides descriptive, exploratory CFQ-R-based HRQoL data in the CFTR modulator era. The findings suggest more favorable respiratory HRQoL among currently treated respondents, while supporting continued multidimensional patient-reported outcome monitoring in Romanian CF care.