Cystic Fibrosis in Iran

Abstract

Background: Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aims to compare the demographic, clinical and paraclinical data of CF patients. Methods: This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. Forced expiratory volume in one second (FEV1) was measured in 174 patients. The demographic, clinical and laboratory data of patients were recorded. Results: Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean (standard deviation) age of patients was 7.1 (5.7) years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. Among patients in whom FEV1 was measured, mild, moderate, and severe involvement were documented in 62%, 33%, and 5% of cases, respectively. Cultures of respiratory secretions were reported to be positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) used oral azithromycin for prophylaxis. Gastro-esophageal reflux disease (35%), osteopenia (22%) and nasal polyps (13%) were the most common complications in the study population. Conclusion: Regarding the complex nature of cystic fibrosis and the necessity of constant monitoring of patients during the life-span, the demographic, clinical and laboratory analysis of patients and registering and standardization of patients’ data, can be a major step in the better understanding of the disease and thereby increasing the quality of life and life expectancy in the affected population.