First cystic fibrosis patient registry annual data report-cystic fibrosis foundation of Iran

Abstract

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 aimed to compare the demographic, clinical and paraclinical data of CF patients. 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. For each patient, the forced expiratory volume in one second (FEV1) was measured, and the comparative demographic, clinical, and laboratory data of patients were recorded. Overall, 59 of studied patients were boys (n=102) and 41 were girls (n=72). The mean patient age (and standard deviations) 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. The patients in this study were classified based on the FEV1 into mild (62), moderate (33) and severe (5), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23 and 16 of cases, respectively. In total, 61 of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35). Regarding the complex nature of CF and the necessity of constant monitoring of patients during the life-span, the comparative 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. © 2019 Tehran University of Medical Sciences. All rights reserved.