Estimation of Incidence and Evaluation of Clinical and Paraclinical Features of Cystic Fibrosis: A Rare Disease in Kurdistan Province between 2010 and 2020

Abstract

Background and Aim: Cystic fibrosis is a rare genetic disease which causes widespread inflammation in the body and has a poor prognosis due to various complications, especially respiratory system involvement. The aim of this study was to estimate the incidence of cystic fibrosis and determine its epidemiological, clinical and paraclinical features in Kurdistan province, Iran, between 2010 and 2020. Materials and Methods: This cross-sectional study included cystic fibrosis patients admitted to Besat Hospital in Sanandaj (as the only referral specialized center for pediatric diseases in Kurdistan Province) between 2010 and 2011. Epidemiological, clinical and paraclinical data of the patients were collected by a checklist. Using SPSS software version 22, data were analyzed by using Mann-Whitney test and Spearman correlation coefficient. Results: During the study period, we found 19 patients with definite diagnosis of cystic fibrosis, 14 (73.7%) male and 5 (26.3%) female, with mean age ± standard deviation of 11.10 ± 9.7. Based on the data of the birth rate in kurdistan Province during the study period, the incidence of cystic fibrosis in Kurdistan Province was estimated to be one in 15,000 live births per year. 4 patients (21.1%), had positive family history. The main symptoms of these patients were steatorrhea (94.7%), cough (89.5%), dyspnea (78.9%), growth disorder (73.7%), heart disorders (63.2%), jaundice (57.9%) and finger clubbing (36.8%). Conclusion: Although cystic fibrosis is not very common in this province, identifying at-risk parents and early diagnosis of the disease can be beneficial for increasing life expectancy and quality of life and reducing health costs.

Keywords

• cystic fibrosis
• genetic disorder
• incidence
• kurdistan