| Abstract | Background: Cystic fibrosis (CF) is autosomal recessive genetic disorder caused by mutations in the CFTR gene. It leads to multisystem complications such as persistent pulmonary infections, pancreatic insufficiency with diabetes, and liver damage, significantly decreasing the quality of life and contributing to morbidity and mortality. This study aims to investigate the prevalence and age of diagnosis of cystic fibrosis-related diabetes (CFRD) in Croatia and explore the connection between the age of CFRD diagnosis, insulin therapy, and body mass index (BMI).
Participants and Methods: This retrospective study analyzes the medical data of 123 patients diagnosed with CF and followed at the Croatian Referral Centre of the Ministry of Health for cystic fibrosis. Data were retrieved from the hospital information system. Among the 123 patients, 17 (6 males, 11 females) were identified with CFRD. The median age was 23 years (range 13-38, SD
6,91), and the median BMI was 22.73 (range 13.55-29.59 SD 3,61) kg/m².
Results: The prevalence of CFRD was 4.49% in the pediatric CF population and 38.24% in the adult CF population. The median age of CFRD diagnosis was 13 years (range 9-24, SD 4.43), with the majority diagnosed between 10 and 15 years (p=0.021; p<0.05). An earlier age of CFRD diagnosis (9-13 years) was associated with higher insulin doses required for therapy, peaking at
age 10 with an average of 1.32 IU/kg. Additionally, those diagnosed at a younger age (4-15 years) had a notably lower mean BMI.
Conclusion: This first study investigating CFRD in Croatia establishes that the median age of diagnosis is predominantly between 10 and 15 years. The prevalence of CFRD aligns with findings from other research. An earlier age of CFRD diagnosis is associated with higher insulin doses required for therapy and a lower BMI, highlighting the importance of early detection and
management of the disease to improve the health outcomes of CF patients. |
| Abstract (croatian) | Uvod: Cistična fibroza (CF) je autosomno recesivna genetska bolest uzrokovana mutacijom CFTR gena. CF dovodi do multisistemskih komplikacija kao što su učestale plućne infekcije, insuficijencija rada gušterače s dijabetesom te oštećenje jetre. CF značajno smanjuje kvalitetu života te pridonoseći morbiditetu i mortalitetu. Cilj ove studije je istražiti prevalenciju i dob dijagnoze dijabetesa povezanog s cističnom fibrozom (CFRD) u Hrvatskoj te istražiti povezanost između dobi dijagnoze CFRD-a, terapije inzulinom i indeksa tjelesne mase (BMI).
Ispitanici i metode: Radilo se o retrospektivnoj studiji u kojoj su analizirani medicinski podatci 123 bolesnika s dijagnozom CF-a koji su praćeni u Referalnom centru Ministarstva zdravstva Republike Hrvatske za cističnu fibrozu. Podaci su prikupljeni iz bolničkog informacijskog sustava. Identificirano je ukupno 17 bolesnika (6 muškaraca, 11 žena) s CFRD-om. Medijan dobi bio je 23 godine (raspon 13-38, SD 6,91), a medijan BMI bio je 22,73 (raspon 13,55-29,59, SD 3,61) kg/m².
Rezultati: Prevalencija CFRD-a bila je 4,49% u pedijatrijskoj populaciji i 38,24% u odrasloj populaciji s CF-om. Medijan dobi dijagnoze CFRD-a bio je 13 godina (raspon 9-24, SD 4,43), s većinom dijagnosticiranih između 10 i 15 godina (p=0,021; p<0,05). Ranija dob dijagnoze CFRDa (9-13 godina) bila je povezana s većim dozama inzulina u liječenju CFRD-a, s vrhuncem u dobi od 10 godina s prosjekom od 1,32 IU/kg. Osim toga, oni dijagnosticirani u mlađoj dobi (4-15 godina) imali su značajno niži prosječni BMI.
Zaključak: U studiji u kojoj je po prvi puta evaluiran CFRD u Hrvatskoj ustanovljeno je da se dijagnoza postavlja najčešće u dobi između 10 i 15 godina. Prevalencija u hrvatskoj populaciji s CF-om slična je onima u drugima istraživanjima. Ranija dob dijagnoze CFRD-a povezana je s većim dozama inzulina i nižim BMI, što ukazuje na važnost ranog otkrivanja i liječenja bolesti kako bi se poboljšali zdravstveni ishodi pacijenata s CF-om. |
