Bronşiectaziile la copii cu fibroza chistică

Abstract

Cystic fibrosis is the most common genetic disease with autosomal recessive way of transmission. It is characterised by exocrine glands dysfunction. Severe progressive pulmonary problems are noted in most patients with cystic fibrosis. In this work there were evaluated clinico-imagistic peculiarities of the bronchiectasis in 40 children with cystic fibrosis. The mean age of patients was 7.07±0.81 years and the mean age of diagnosis was 1.11±0.24 years. The frequency of bronchiectasis presented a significant (r2 =0.97, p<0.01) increase with the age of patients with cystic fibrosis.

Fibroza chistică este cea mai frecventă maladie genetică cu transmitere autozomal recesivă, care se caracterizează prin disfuncţie generalizată a glandelor exocrine. Majoritatea pacientilor cu fibroza chistica dezvolta patologie pulmonară progresivă. A fost realizat un studiu clinicoimagistic a bronşiectaziilor la 40 copii cu fibroză chistică. Pacienţii au prezentat o vârstă medie de 7,07±0,81 ani, iar vârsta medie de diagnostic al fibrozei chistice a constituit 1,11±0,24 ani. Frecvenţa dezvoltării bronşiectaziilor a prezentat un indice de corelaţie foarte înalt (r2 =0,97, p<0,01) cu vârsta pacienţilor.