Cystic Fibrosis Prevalence among a Group of High-Risk Iranian Children

Knowledge about cystic fibrosis (CF) in Iran is very limited. The objective of this study was to determine the prevalence of CF among a group of high risk children with suggestive clinical features in Tehran Pediatric Medical Center, Tehran, Iran. A total number of 505 patients aged 0-15 years participated in this study. Subjects presented with one or more of the following symptoms were asked to participate in the study: chronic or recurrent respiratory symptoms, gastrointestinal symptoms such as rectal prolapsed, steatorrhea, hepatobiliary disease such as prolonged jaundice, failure to thrive, hyperglycemia and glycosuria, hypochloremic metabolic alkalosis, hypoprothrombinemia, anemia, or edema. Children with positive family history of CF were also included. Patients were screened using pilocarpine iontophoresis to collect sweat. Chemical analysis of sweat chloride content was performed by classic Gibson and Cooke technique. Of 505 patients, 89 (17.6%) had positive sweat chloride screening. In 5 patients (1%), cystic fibrosis transmembrane regulator (CFTR) mutation analysis was required to confirm CF. The study results suggested that CF is more common in Iran than previously anticipated. Larger studies are warranted to identify the incidence, molecular basis, and clinical pattern of CF in the Iranian population.