Results: The study included 9 female and 14 male patients with an age range of 5-23 years (mean: 13.42 years). Bronchiectasis (100%) and peribronchial wall thickening (100%) were the most frequent CT abnormalities. Mucus plugging, air trapping and parenchymal involvements were respectively seen in 95.7%, 91.3% and 47.8% of patients. The overall CT score for all patients was 57.6±24.2 (means±SD). The results of pulmonary function test http://www.selleckchem.com/products/z-vad-fmk.html showed a restrictive pattern; however, in 5.3% of the patients PFT was normal. The overall Shwachman-Kulczycki score was 53.48±13.8. There was a significantly (P=0.015) negative correlation between the total CT score and Shwachman-Kulczycki score; however, there was no significant

correlation between total CT score Inhibitors,research,lifescience,medical and the results of PFT Inhibitors,research,lifescience,medical (P=0.481) Conclusion: The Brody’s scoring system for high resolution computed tomography seems to be a sensitive and efficient method to evaluate the progression of CF, and can be more reliable when we combine the CT scores with clinical parameters. Key Words: Clinical status, pulmonary function test, cystic fibrosis Introduction Cystic fibrosis (CF) is the most common fatal genetic disorder in white population.1,2 Due to new and restrict Inhibitors,research,lifescience,medical modalities in the treatment of CF patients, their survival has increased. However, CF is still responsible for major complications, which increase the mortality and morbidity rates in such

patients.3 The most common cause of mortality in CF patients is chronic pulmonary disease, which is the consequence of persistent Inhibitors,research,lifescience,medical infections and inflammations.1,2,4 To evaluate the pulmonary status in CF, a number of diagnostic procedures including chest radiography, high resolution computed tomography (HRCT), sputum culture

and pulmonary function test are considered.1,2,4-7 Since CT Scan was found to be one of the best evaluation tools for cystic fibrosis progression, CT scoring system was proposed to make the evaluation more effective.8-11 A computed tomography scoring system is a tool to describe the abnormalities found by CT scan.12 The scoring system was introduced by Bhalla and colleagues 12. Since then, a number of other scoring systems Inhibitors,research,lifescience,medical have been proposed by Helbich,13,14 Santamaria,15 and Anacetrapib Brody,16 and their colleagues. Brody’s scoring system is a lobar scoring system, which assigns a score to each lobe separately. This scoring system describes the following morphologic changes: bronchiectasis, peri-bronchial wall thickening, mucus plugging, air trapping and parenchymal involvement.16 Although different studies were conducted to show the usefulness of Brody CT scoring system in the assessment of the progression of the disease,16-19 possible correlation between the Brody scoring system and clinical status in patients with CF has not been examined. Therefore, the present study was designed to examine the correlation of the Brody scoring system with clinical parameters and pulmonary function test (PFT) in pediatric patients with CF.