Naslov
Insight into the outcomes of Croatian patients with cystic fibrosis
(429 Insight into the outcomes of Croatian patients with cystic fibrosis)

Autori
Brajcinović, M. ; T-Drinković, D. ; Vukić-Dugac, A. ; Tjesić-D, D.

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Skup
ECFS Conference Seville 2017

Mjesto i datum
Sevilla, Španjolska, 07.06.2017. - 10.06.2017

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
cystic fibrosis, outcomes

Sažetak
Objectives: Croatia is not in the European Cystic Fibrosis Patient Registry since 2008 ; hence there are currently no relevant demographic or clinical data on our patients with cystic fibrosis (CF). This retrospective crosssectional study describes basic characteristics of patients older than 6 y followed from Jan-Dec 2015. in a CF Unit which takes care of > 50% of all (≈ 160) CF Croatian patients. Methods: We analyzed nutritional status, respiratory outcome, chronic Pseudomonas aeruginosa (PA) infection and their correlations using descriptive statistics, Spearman and Mann-Whitney tests. Results: We identified 39 patients with data on FEV1% predicted value (pv), height and weight measured at the date of best FEV1 and at least 4 sputum samples collected. There were 28 pediatric patients (6–19 y) and 11 adults (19 y 6 mo–30 y 11 mo) ; median age 15 y 11 mo ; 22/39 female. All have at least one copy of F508del ; 26/39 are homozygous. Median age of diagnosis is 4 months. Median BMI Percentile by Age (BMFA%) in patients 6–19 y is 46.55. However, there is a trend of decline with age: in 6–12 y olds, BMFA% is 48.7 ; in 13–19 y olds, BMFA% is 33.6. Median BMI in CF adults is 21 (range 18.7–25.9). In the whole study group, the median FEV1%pv is 77.5 ± 31.28. Lung function significantly decreases with age (r = −0.713, p < 0.0001) and falls below 80% after 14 y of age. In children, nutritional status and lung function correlate positively (r = 0.409, p = 0.031). This was not confirmed in adults, probably due to a small sample with large variability. Chronic PA infection is present in all adults and 7/28 children. The median age of noncolonized patients is 9.4 y (range 6–13.5). Lung function is significantly worse in chronically colonized patients (mean FEV1%pv 61.0 vs 91.6 ; p = 0.0019). Conclusion: This is an insight into the outcomes of Croatian CF patients, accentuating the urge for health care reorganization and a starting point to improve patients’ perspectives.

Izvorni jezik
Engleski

POVEZANOST RADA