Cystic Fibrosis Patients Evaluation in the Last Decades in a Referral Center, Tehran-Iran

Background cystic fibrosis (cf) is the most prevalent lethal chronic genetic and multivariate disease, widespread in iranian population. the aim of this study was to evaluate the condition of patients with cf from various dimensions in the last decades in iran. materials and methods in this cross-sectional study the data were collected from disease registry database of cf patients in pediatric respiratory disease research center in masih daneshvari, tehran, iran. the patients were divided into female and male. the data were collected based on province, birthplace province, marital status, and degree of education, parental consanguinity ratio, age, and age of onset of symptoms, age of onset of diagnosis and body mass index (bmi). results majority of patients (n=169, 59.3%) were male, born in tehran province (n=81, 28.4%), and live in tehran province (n=102, 35.8%). majority of parents (61.1%, n=174) had consanguineous relation and the rest (n=94, 33%) did not have any consanguineous relation. results show that mean of age, age of diagnosis, mean for expression of first symptoms and mean of time interval between the onset of symptoms and the diagnosis of the disease were 14.52, 2.51, 6.57, and 4.07 years, respectively. results showed that high percentage of patients (n=191, 67%) had lower bmi (<18.50), and low percentage (n=3, 1.1%) showed higher bmi (>25). correlation coefficient also showed positive relation between start age of symptoms and diagnosis age of disease. conclusion in iran, cf is a multisystem disease and age for its expression is different. since mean for time interval between the onset of symptoms and the diagnosis of the disease is 4.07 years, it could be recommended to perform the phenotypic and genotypic test at birth.