Predictors of deterioration of lung function in Polish children with cystic fibrosis

Abstract

IntroductionSeverity of lung disease varies in patients with the same CFTR genotype. It suggests that other factors affect the severity of cystic fibrosis (CF). The aim of the study was to identify risk factors that determine lung function decline in Polish cystic fibrosis children.Material and methodsThe follow-up time was no less than 5 years of respiratory status observation based on the forced expiratory volume in 1 s value (FEV1). The socio-economic data, perinatal interview, presence of meconium ileus (MI), time of CF diagnosis, initiation of tobramycin inhalation solution (TIS), pancreatic function, sensitization to Aspergillus fumigatus, presence of impaired glucose tolerance (IGT) or diabetes mellitus, chronic bacterial colonization and number of exacerbations and hospitalizations were assessed.ResultsThe mean age of 61 included children was 13.3 ±7.6 years. Delta F508 homozygosity was detected in 45.9%, 44.3% were delta F508 heterozygous, and 9.8% had other genotypes. FEV1 decline was observed among 20% of patients; the rest of the patients presented stable values of FEV1 during at least 5 years of observation. The most significant predictors related to the decline of FEV1 were presentation of MI (p = 0.0344), IGT (p = 0.0227), number of exacerbations (p = 0.0288), and early Pseudomonas aeruginosa (PA) chronic colonization (p = 0.0165) followed by late TIS initiation after the first detection of PA (p=0.0071). Neither time of diagnosis nor type of CFTR mutation was statistically significant as a predictor of lung deterioration.ConclusionsThe presence of MI, IGT, chronic PA colonization, and number of exacerbations are risk factors for lung function deterioration.

Diabetes and Prediabetes in Children With Cystic Fibrosis: A Systematic Review of the Literature and Recommendations of the Italian Society for Pediatric Endocrinology and Diabetes (ISPED)

“…Prediabetes was one of the most relevant predictors of deterioration of lung function defined as a significant decrease in FEV1 predicted value, during a 5 year-follow-up ( 17 , 18 ) (evidence Moderate).…”

“…Among prediabetes, IGT represents one of the most relevant predictors of the decline in pulmonary function ( 17 ) ( 18 ) and both CFRD and IGT have been recognized as independent risk factors for pulmonary exacerbations and colonization by common CF pathogens, in particular by Pseudomonas Aeruginosa , and its form of infection characterized by multi-resistance to antibiotic therapy and co-infection with other pathogens ( 11 , 16 , 19 , 21 – 23 , 25 ).…”

Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs

“…A recent Polish study found that early P. aeruginosa chronic colonization was a predictor of lung function deterioration (p=0.0165) among other risk factors (presentation of meconium ileus, impaired glucose tolerance, number of exacerbations), contrary to time of diagnosis and type of CFTR mutation. 30 In Tunisia, lung function tests are difficult to perform in children due to their limited availability. Monitoring of the microbiology sputum examinations is often irregular and the analysis is limited to the exacerbation period.…”

Diabetes and prediabetes in children with cystic fibrosis

“…Before we begin to describe the different treatment options for CFRD and cystic fibrosis-associated prediabetes, an important premise needs to be made: the therapy of the different forms of glucose derangement has a favourable impact not only on strictly metabolic outcomes but also, and above all, on the morbidity and complications associated with cystic fibrosis. In particular, a favourable impact on pulmonary function, growth, nutritional status and the recurrence of respiratory infections has been demonstrated [11][12][13].…”