characteristics of patients with cystic fibrosis: experience in a large referral children’s hospital in tehran, iran

â  â  b ackground cystic fibrosis (cf) is an autosomal recessive disease caused by a cf trans-membrane regulator (cftr) defect. its prevalence is 1:2500 in caucasians, 1:15300 among african americans and is rare in southâ­east asia. the present study aims to review demographic data, clinical manifestations and laboratory findings of iranian children diagnosed with cf who referred to a children’s hospital medical center in tehran, iran during a ten-year period. me t hod s in a retrospective study from 1991-2000, all hospitalized patients with documented cf were reviewed. diagnosis was based on clinical findings and sweat chloride levels above 60 meq/l. re s ul ts a total of 233 patients [females: 91 (39.1%), males: 142 (60.9%)] were enrolled. the onset of symptoms was before the first month of life in 12.1%, between 1-6 months of age in 75.1%, and between 6-12 months of age in 6.9% of patients. consanguinity of parents was present in 42.5% of patients. respiratory (81.5%) and gastrointestinal (73.4%) symptoms, in addition to growth retardation were the most common presentations eighty-eight percent of patients weighted below the fifth percentile.â  of the 207 chest radiographs performed, the most frequent finding was hyper-aeration associated with pneumonia. among 138 patients in whom barium swallows were performed, 102 (74%) had gastroâ­esophageal reflux. a total of 27 patients expired, mostly from respiraâ­tory failure (96.3%). con c lu s ion cf is not a rare disease in iran. we suggest early diagnosis and appropriate maintenance therapy for improving morbidity and mortalâ­ity amongst cf patients.