The Turkish Journal of Pediatrics
2010 , Vol 52 , Num 2
The Clinical and Laboratory Manifestations of Iranian Patients with Cystic Fibrosis
1Department of Pediatrics, Pediatrics Center of Excellence, Children’s Medical Center, and 2Growth and Development
Research Center, Tehran University of Medical Sciences, Tehran, Iran
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.
Keywords : cystic fibrosis, respiratory infection, Iran.