Caracteristici ale infecţiei bacteriene endobronşice în Fibroza Chistica: analiză paralelă cu un studiu clinic efectuat pe pacienţi pediatrici în două centre de Fibroză Chistică în România în perioada 1999-2006 | [Caracteristics of bacterial ...]

Abstract

Cystic Fibrosis (CF) is an autosomal recessive caused by mutations in a single gene on the long arm of chromosome 7 that encodes the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein. Despite advances in understanding the molecular basis and pathophysiology of this disorder, it remains the most common life-shortening genetic disorder in the white population. Due to abnormal CFTR, the clinical consequences are represented by a multisystem disease involving predominantly the respiratory, gastrointestinal, hepatobiliary, and male reproductive tract. Patients are susceptible to recurrent respiratory infections with a variety of microorganisms especially Pseudomonas aeruginosa, Burkholderia cepacia complex, Staphylococcus aureus, Haemophilus influenzae, and Aspergillus species. The treatment of lung disease represents the mainstay of therapy along with improvement of nutritional status, enzyme replacement therapy, and physiotherapy. In order to prolong the life span and improve the quality of life of CF patients, treatment of patients has to be assured by multidisciplinary teams. The lung disease, a marker of outcome has to be treated early and aggressively. The characteristics of respiratory infection in children with CF represent the subject of this paper along with the presentation of results of the study of patients followed up in 2 CF centres during the period 1999-2006..