Sažetak sa skupa
Nasal polyposis in cystic fibrosis: experience from CF Center, UHC Zagreb
Katarina Đurić Vuković
orcid.org/0000-0003-4763-6711
; KBC Zagreb, Klinika za bolesti uha, nosa i grla i kirurgiju glave i vrata
Sunčica Kružić
; Opća bolnica Vinkovci, Odjel za otorinolaringologiju
Ivan Bambir
; KBC Zagreb, Klinika za pedijatriju
Gorazd Poje
; KBC Zagreb, Klinika za bolesti uha, nosa i grla i kirurgiju glave i vrata
Andrea Vukić Dugac
; KBC Zagreb, Klinika za plućne bolesti
Duška Tješić Drinković
; KBC Zagreb, Klinika za pedijatriju
Sažetak
Cystic fibrosis (CF) is a lethal autosomal recessive disease, caused by mutations in the CF
transmembrane conductance regulator gene (CFTR). CFTR mutations affect epithelial cells in the lungs,
sinuses, pancreas, liver, kidneys, intestine and sweat glands, causing abnormally viscous mucus production,
thickening of digestive fluids and salty sweat. The consequences for the respiratory system are mucus buildup, decreased mucociliary clearance and tissue inflammation. A change in microbioma follows, with S. aureus
and P. aeruginosa being prevalent in most patients, as they have the capacity of biofilm formation causing
chronic colonization. This represents the basis for recurrent infection. At the same time, pancreatic
insufficiency leads to malabsorption of fat-soluble vitamins, i.g. vitamin D3, a powerful immunomodulator
implicated in both pulmonary and sinus pathophysiology. CF is marked by a high incidence of nasal polyposis,
even in the pediatric population and ENT follow-up is mandatory. CF nasal polyposis is a distinctive form of
nasal polyposis and its treatment and follow up present many challenges.
CF affects one out of every 3000 newborns. The total number of patients with CF in Croatia is 175,
and the majority of them now refer to our CF Center of Zagreb University Center where they are approached
by a multidisciplinary team: both pediatric and adult pulmologists, gastroenterologists, endocrinologists,
rhinologists, microbiologists and nutritionists. We aimed to review our experience with CF patients from a
rhinologist point of view and present the prevalence and specifics of nasal polyposis in cystic fibrosis patients
from our CF Center.
Ključne riječi
nasal polyposis; cystic fibrosis; sinus surgery; vitamin D3 deficiency
Hrčak ID:
273313
URI
Datum izdavanja:
27.2.2022.
Posjeta: 346 *