Cystic fibrosis prevalence among a group of high‑risk children in the main referral children hospital in Iran
نویسندگان
1 Department of Pediatric Pulmonology, Child Growth and Development Research Center, Faculty of Medicine, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan
2 Department of Pediatric Pulmonology, Child Growth and Development Research Center, Faculty of Medicine, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan
3 Department of Pediatric Pulmonology, Child Growth and Development Research Center, Faculty of Medicine, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan
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5 Department of Pediatric Pulmonology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
6 Department of Pediatric Pulmonology, Child Growth and Development Research Center, Faculty of Medicine, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan,
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doi
چکیده
BACKGROUND: Knowledge about cystic fibrosis (CF) in Iran is very limited. The objective of thisstudy was to determine the prevalence of CF among a group of high‑risk children with suggestiveclinical features in the main referral hospital in Iran.MATERIALS AND METHODS: This study children consisted of 505 patients who had presented withone or more of the following symptoms: chronic or recurrent respiratory symptoms, gastrointestinalsymptoms as rectal prolapse, steatorrhea, hepatobiliary disease as prolonged jaundice, failure tothrive, hyperglycemia and glycosuria, hypochloremic metabolic alkalosis, hypoprothrombinemia,anemia or edema, and positive family history of CF. Patients were screened using pilocarpineiontophoresis to collect sweat and chemical analysis of its chloride content with classic Gibson andCooke technique.RESULTS: Of 505 patients, 89 (17.6%) had positive sweat chloride screening test. Five (1%)patients had required cystic fibrosis transmembrane conductive regulator protein mutation analysisto confirm CF.CONCLUSION: Our findings suggest that in Iran, CF is more common than what previouslyanticipated. Larger studies are warranted to identify the incidence, molecular basis, and clinicalpattern of CF in the Iranian population.
