| Abstract | Cistična je fibroza autosomno recesivna nasljedna multisistemna bolest od koje se u Hrvatskoj danas liječi oko 150 bolesnika. Ponajviše pogađa probavni i dišni sustav, stoga su plućna funkcija ( FEV1 - forsirani ekspiratorni volumen u 1 sekundi, % očekivane vrijednosti) i uhranjenost najčešće korišteni parametri za procjenu stanja bolesti. Plućna je funkcija osnovni prediktor smrti te je dugoročno očuvanje plućne funkcije od životne važnosti za oboljele od cistične fibroze. ----- Cilj ove presječne studije bio je dobiti relevantne podatke za oboljele od cistične fibroze u Hrvatskoj te opisati eventualnu povezanost uhranjenosti i plućne funkcije, na što upućuju brojna dosadašnja istraživanja. U istraživanje je uključeno 39 ispitanika starijih od 6 godina (raspon dobi 6 g – 30 g 11 mj.) koji su se tokom 2015. liječili u KBC-u Zagreb. Analizirana je njihova dob, plućna funkcija i prisustvo kronične kolonizacije dišnog puta s Pseudomonasom aeruginosa.
Uhranjenost ispitanika mlađih od 19 godina (N=28) zadovoljavajuća je uz medijan 46,55 percentila BMFA (eng. body mass index for age, indeks tjelesne mase za dob). U skupini bolesnika starijih od 19 godina medijan ITM (indeks tjelesne mase) iznosi 21. Plućna funkcija u cijelom promatranom uzorku značajno pada s dobi (snažna negativna korelacija FEV1 % očekivane vrijednosti i dobi, Spearman r=- 0,713, p<0,0001). Također je utvrđena statistički značajno lošija plućna funkcija (Mann-Whitney p=0,0019) u skupini bolesnika kronično koloniziranih s Pseudomonasom aeruginosa (N= 18, prosječan FEV1 61%) u odnosu na nekolonizirane (N = 21, prosječan FEV1 91,6%). U skupini bolesnika mlađih od 19 godina postoji i statistički značajna pozitivna korelacija uhranjenosti i plućne funkcije (Spearman r=0,409, p=0,031), što se nije potvrdilo u bolesnika starijih od 19 g, vjerojatno zbog malog uzorka velike varijabilnosti. ----- Zaključno, rezultati ukazuju na važnost nutricionističke potpore u cističnoj fibrozi u svim dobnim skupinama, a sa ciljem što duljeg održanja dobre plućne funkcije. Važno je o ovoj povezanosti osvijestiti medicinsko osoblje, ali i bolesnike same s obzirom da mogu aktivno utjecati na vlastitu uhranjenost. |
| Abstract (english) | Cystic fibrosis is an autosomal recessive inherited multisystem disease recognized in about 150 patients today in Croatia. It mainly affects the digestive and respiratory system, so the pulmonary function (FEV1 - forced expiratory volume in 1 second, % predicted) and nutritional status are the most commonly used parameters to assess the state of the disease. Pulmonary function is a primary predictor of mortality and long - term preservation of lung function is of vital importance for patients with cystic fibrosis. ----- The aim of this cross-sectional study was to obtain relevant information for patients with cystic fibrosis in Croatia and describe a possible connection between nutritional status and lung function, as indicated in numerous previous studies. This study included 39 patients aged 6 years and older (age range 6 g - 30 years 11 months) who were treated at the University Hospital Center Zagreb in 2015. Age, pulmonary function and the presence of chronic Pseudomonas aeruginosa colonization in the airways were analyzed.
Nutritional status of patients under the age of 19 (N = 28) is satisfactory with median BMFA (body mass index for age) of 46.55 percentile. In patients aged 19 years and older, the median BMI (body mass index) is 21. In the whole study group pulmonary function significantly decreases with age (strong negative correlation between FEV1 % predicted and age, Spearman r = - 0.713, p <0.0001). Statistically significant (Mann-Whitney p = 0.0019) worse lung function was found in patients chronically colonized with Pseudomonas aeruginosa (n = 18, mean FEV1 61%) when compared to those who were not colonized (N = 21, mean FEV1 91.6 %). In the group of patients under the age of 19 there is a statistically significant positive correlation between nutritional status and lung function (Spearman r = 0.409, p = 0.031), which was not confirmed in patients aged 19 and older, probably due to a small sample with large variability. In conclusion, these results indicate the importance of nutritional support in cystic fibrosis in all age groups, with the aim of maintaining good lung function as long as possible. It is important to raise awareness of this link among medical staff but also the patients themselves, as they can actively influence their own nutritional status. |
