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: Cystic fibrosis(CF )liver disease is an important developing complication, while CF lung disease influence significantly the outcome. As CF is characterized by obstructive respiratory disfunction and CF liver disease may associate restrictive dysfunction, we aimed to evaluate the lung function of these children. Methods: The cross sectional study included patients with cystic fibrosis liver disease(CFLD), F508 del homozygous, age 10-18 years. They were routinely followed-up by clinical assessment, liver biochemical tests, ultrasound examinations (US) every 3 months. Every visit, spirometry, LCI through MBW(multiple breath washout ) was performed, except exacerbation. Results: Of 74 screened patients, 25 children were diagnosed with CFLD, 40.7% were diagnosed with severe CFLD, median age 15.6 years. Restrictive dysfunction was found in 21% of patients, caused by hepatomegaly; mixed respiratory dysfunction was found in 28%. A good correlation between LCI with FEF 25-75%(r=0.87) with an average of 31.6% and a median FEV1 =64.7%. was found. In moderate CFLD, there was a disagreement between a low median FEF 25-75 % (44.8%) and good FEV1 (7mean 6.5%). We found that distal obstruction was present in CFLD, almost all patients having lower than normal FEF 25-75%. No correlation between the LCI values and presence CFLD was found. Conclusion: Lung function is impaired in children with CF liver disease, without a specific pattern. In patients with more severe lung disease expressed by obstruction, CFLD is not an independent risk factor. Restrictive respiratory dysfunction was found only in severe CFLD.