For each question, a structured review of the literature was performed using the Medline database in order to identify the studies with the methodological design most appropriate to answering the question. Mc Coy KS, Quittner AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB. An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis.
Key words: Cystic fibrosis/diagnosis; Cystic fibrosis/drug therapy; Pseudomonas aeruginosa; Evidence-based medicine Patients with CF are born with structurally normal lungs, but develop a progressive respiratory disease with recurrent chronic infections that result in the formation of bronchiectasis and lead to respiratory failure, which is the leading cause of death in these subjects.
The basic defect in CF is related to chlorine transport through epithelial cell membranes by the cystic fibrosis transmembrane conductance regulator (CFTR) protein, the dysfunction of which was identified as being the principal mechanism of the disease in 1989.
There are more than 1,500 described mutations in the CFTR gene sequence, but most of them have very low prevalence, the Î"F508 mutation (deletion of phenylalanine residue at position 508) being the most prevalent worldwide.
Patients with CF are peculiarly susceptible to infection and colonization of the respiratory tract with pathogens, such as Staphylococcus aureus, Haemophilus influenza, and glucose-nonfermenting gram-negative bacilli, including Pseudomonas aeruginosa, Burkholderia cepacia complex, and Stenotrophomonas maltophilia, among others.
The prevalence of these pathogens varies with age, with S.
aureus infections usually occurring early (generally in the first months of life) and pathogens such as P.
aeruginosa tending to appear a little later, although this sequence of infections are greatly influenced by therapeutic and microbiological surveillance practices, as well as by hospitalizations, exposure to other patients with CF, and environmental conditions that have yet to be defined. Murphy TD, Anbar RD, Lester LA, Nasr SZ, Nickerson B, Van Devanter DR, et al.
ABSTRACT Evidence-based techniques have been increasingly used in the creation of clinical guidelines and the development of recommendations for medical practice.
The use of levels of evidence allows the reader to identify the quality of scientific information that supports the recommendations made by experts. Geller DE, Flume PA, Staab D, Fischer R, Loutit JS, Conrad DJ, et al.
The objective of this review was to address current concepts related to the clinical impact, diagnosis, and treatment of infections in patients with cystic fibrosis. Konstan MW, Flume PA, Kappler M, Chiron R, Higgins M, Brockhaus F, et al. Levofloxacin inhalation solution (MP-376) in patients with cystic fibrosis with Pseudomonas aeruginosa.
For the preparation of this review, the authors defined a group of questions that would be answered in accordance with the principles of PICO–an acronym based on questions regarding the Patients of interest, Intervention being studied, Comparison of the intervention, and Outcome of interest. Oermann CM, Retsch-Bogart GZ, Quittner AL, Gibson RL, Mc Coy KS, Montgomery AB, et al. Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial.