title: The clinical and laboratory manifestations of Iranian patients with cystic fibrosis.
abstract: Cystic fibrosis (CF) is a hereditary disease, characterized by chronic pulmonary disease, pancreatic insufficiency and abnormal electrolytes in the sweat. In order to evaluate the clinical manifestations and laboratory findings of Iranian children with CF during a 10-year period, 243 CF patients, with a median age of 5 months, were investigated in this study. The most common manifestations were gastrointestinal disorders and respiratory manifestations. Cough was the most common symptom, followed by malnutrition, diarrhea, respiratory distress, and vomiting. The frequency of these findings after treatment was significantly decreased in comparison with the period before diagnosis. During the mean follow-up of 40.9 months, seven cases died due to severe infections. Cystic fibrosis as a common genetic disorder should be considered in any child with recurrent gastrointestinal and respiratory manifestations, since delayed diagnosis could lead to severe complications and even death in this group of patients.
authors.affiliation: Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, Iran.
authors.forename: Gholamhossein
authors.initials: G
authors.lastname: Fallahi
authors.forename: Mehri
authors.initials: M
authors.lastname: Najafi
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Farhmand
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Bazvand
authors.forename: Maedeh
authors.initials: M
authors.lastname: Ahmadi
authors.forename: Faezeh
authors.initials: F
authors.lastname: Ahmadi
authors.forename: Kambiz
authors.initials: K
authors.lastname: Eftekhari
authors.forename: Ahmad
authors.initials: A
authors.lastname: Khodad
authors.forename: Farzaneh
authors.initials: F
authors.lastname: Motamed
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Khatami
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007492:Iran / Q000453:epidemiology; D008297:Male; D012307:Risk Factors; D055815:Young Adult