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Methods: This longitudinal study was conducted in 2017-2021 on 250 CF patients referred to the CF clinic of Akbar hospital in Mashhad. For each patient, the comparative demographic, clinical and laboratory data of patients were recorded.

Results: About 54% of studied patients were boys (n= 135) and 46% were girls (n= 115). The mean patients age (SD) was 9.85±6.02 years, with a range of 6 month to 37 years. The mean age of diagnosis was 18.83±38.82 and about 9.6% of patients diagnosed after 5 years. In 75% of patients, family history was positive. The most important clinical presentation at the time of diagnosis was failure to thrives. Other were include Recurrent pneumonia, Steatorrhea and Meconium Ileus. Trend of change in malnutrition showed BMI z score increased significantly from-1.42±1.7 to-. 72±1.33 in 2017 compared with 2021 (p< 0.05), although 19.8% were moderate to severe undernutrition …