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Introduction: Cystic fibrosis (CF) is an inherited disorder that leads to the production of thick, sticky mucus in the lungs and digestive system. CFTR protein modulators, such as Kaftrio and Kalydeco, represent a significant advancement in CF treatment by targeting the underlying molecular defect. Aim: to evaluate the clinical efficacy and safety of CFTR protein modulators in pediatric CF patients at the Clinical Center University of Sarajevo. Materials and methods: eight pediatric patients aged 6 to 18 years with a confirmed CF diagnosis were included. Clinical outcomes such as weight, height, lung function (FEV1), and liver enzyme levels were monitored. Results: all patients showed significant improvements in weight and lung function, with an average FEV1 increase of 13%. Liver enzyme levels normalized in all cases, and no serious adverse events were reported. Conclusion: CFTR protein modulators, particularly Kaftrio and Kalydeco, offer significant clinical benefits for pediatric CF patients, highlighting their potential as a first-line therapy. [ABSTRACT FROM AUTHOR]

The aim of our study was to assess the nutritional status of hospitalized children with cystic fibrosis. We extracted data from the ePINUT surveys. Undernutrition was defined as a body mass index (BMI) of <18.5 according to the International Obesity Task Force cut-off, and the nutritional status goal was defined as a BMI z-score ≥0 SD for children older than 2 years and a weight-for-height z-score ≥0 SD for those younger than 2 years. Undernutrition frequency in the 114 patients with cystic fibrosis was 46% and was higher than in children with other chronic diseases (n = 5863; 30.5%; p = 0.001); 81% of children were below the nutritional status goal. Undernutrition frequency in cystic fibrosis is higher than in other chronic diseases. [ABSTRACT FROM AUTHOR]

Background: Different respiratory sampling methods exist to identify lower airway pathogens in patients with cystic fibrosis (CF). Bronchoalveolar lavage (BAL) is considered the "gold standard.". Because BAL is invasive and cannot be repeated frequently, identification of lower respiratory tract pathogens in CF patients is challenging. Other sampling techniques are sputum, cough, and nasal swabs. The aim of the work: To differentiate between the microbiological results of bronchoalveolar lavage (BAL), sputum and cough swabs in CF children and to evaluate their accuracy in detecting lower respiratory tract organisms. Patients and Methods: Microbiological results detected by these different sampling methods were compared in all enrolled CF patients (n=31). Specificity, sensitivity, positive (PPV), and negative (NPV) predictive values were calculated. Results: Regarding microbiological results, Concordance was found between BAL and sputum as regards Pseudomonas spp., Klebsiella spp. and Enterobacter spp., except for Staph aureus (P 0.039). overall low sensitivity (7.1%) and low accuracy (16.1%) of cough swabs to detect lower airway pathogens [Pseudomonas spp., Staphylococcus aureus spp., Klebsiella spp. and Enterobacter spp.] in children with CF followed by sputum analysis as it showed sensitivity (85.7%) and accuracy (80.6%) while BAL showed highest sensitivity (86.2%) and highest accuracy (82.9%). Conclusion: Our findings suggested that sputum samples showed a good bacteriologic concordance with BAL in detecting lower airway pathogens, While Cough swabs showed the lowest accuracy in detecting the presence of CF pathogens in clinically stable CF children. However, BAL should be performed in symptomatic patients who could not expectorate and had a negative cough swab and sputum sample. [ABSTRACT FROM AUTHOR]

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The study aimed to assess the regional peculiarities of the respiratory profile of children with cystic fibrosis (CF) in the Dnipro region (Ukraine). Methods. Children living in the Dnipro region and aged younger than 18 years old with molecular-genetic confirmation of CF were enrolled in the study. Lung colonization was evaluated using a culture-dependent method. Sputum, mucus from the posterior pharyngeal wall and bronchoalveolar lavage fluid (BALF) were utilized. Results. The Firmicutes phylum was the most common and occupied 54.00 % of the general proportion. On the other hand, the Proteobacteria phylum demonstrated overexpression in CF airways and kept the second rank with 28.87 %. Sorensen's species similarity coefficient showed an allied affinity between the microbial burden of oropharyngeal samples with nasopharyngeal and sputum, QS = 0.61 and 0.91, respectively. However, the species composition within the nasal cavity was distinct from sputum and BALF (QS = 0.47). The primary pathogens in childhood were S. aureus, H. influenza, P. aeruginosa and A. fumigatus. In contrast to gram-negative non-fermenters (GNNF), the prevalence of S. aureus isolates by age had a non-linear character. The commensal microbiota changed negatively with age. Among children under 12 years, the Streptococcus genus was identified in 23.08 % of the samples, but among the age category older than 15 - only in 9.22 %. 11.06 % of S. aureus had small colony variants (SCVs) morphotypes. Isolates of P. aeruginosa with the properties of SCVs were also found in children who underwent prolonged antimicrobial treatment. However, the most prominent was the mucoid phenotype - 34.31 % of isolates. Conclusions. Along with conventional microbiological properties, obligate pathobionts in children with CF exhibited changes, resulting in difficulties in identification. These included auxotrophic modification into SCVs and mucoid transformation. The culture-dependent technique gives crucial data about the profile of pathogens usually associated with CF, although it is sufficiently limited. [ABSTRACT FROM AUTHOR]

Although many Clinical Practice Guidelines (CPGs) have been published for the care of patients with Cystic Fibrosis (CF), including a variety of nutrition recommendations, the quality of these CPGs has never been evaluated. The aim of this study was to compare, review, and critically appraise CPGs for the nutritional management of CF, throughout the lifespan. We searched PubMed, Guidelines International Network (GIN), ECRI Institute, and Guidelines Central for CPGs, with information on the nutritional management of CF. Retrieved CPGs were appraised by three independent reviewers, using the Appraisal of Guidelines, Research and Evaluation II (AGREE II) instrument and checklist. A total of 22 CPGs (seven solely nutrition oriented), by 14 different publishers, were retrieved. The Thoracic Society of Australia and New Zealand CPGs scored the highest overall quality (94.4%), while the Paediatric Gastroenterology Society/Dietitians Association of Australia CPGs had the lowest score (27.8%). Great variation in AGREE II domain-specific scores was observed in all CPGs, suggesting the existence of different strengths and weaknesses. Despite the availability of several CPGs, many appear outdated, lacking rigor, transparency, applicability, and efficiency, while incorporating bias. Considering that CPGs adherence is associated with better outcomes and the need for improving life expectancy in patients with CF, the development of CPGs of better quality is deemed necessary. [ABSTRACT FROM AUTHOR]

The article reports on the use of magnetic resonance imaging (MRI) and newborn screening to detect lung disease progression in children with cystic fibrosis. Topics include microbiological pathogen detection from throat swabs, morphofunctional chest MRI in sedated children and use of analysis of variance to analyze quantitative data.

The article reports on the use of magnetic resonance imaging (MRI) with newborn screening to detect lung disease progression at an early stage in cystic fibrosis (CF). Topics include ability of MRI to track progression of diseases, detection of airway problems in children with CF and use of sedation or distraction techniques.