title: HRCT in cystic fibrosis in patients with CFTR I1234V mutation: Assessment of scoring systems with low dose technique using multidetector system and correlation with pulmonary function tests.
abstract: OBJECTIVE
  Pulmonary changes in patients with cystic fibrosis (CF) with CFTR I1234V mutation have not been extensively documented. Impact of geographic influence on phenotypical expression is largely unknown. This descriptive clinical study presents the high-resolution computed tomography (HRCT) pulmonary findings and computed tomography (CT) scoring with respect to pulmonary function tests (PFT) in a small subset of CF group.
  
  
  METHODS
  We examined 29 patients between 2 and 31 years of age with CFTR I1234V mutation. HRCT and PFT were performed within 2 weeks of each other. Imaging abnormalities on HRCT were documented and analyzed by utilizing the scoring system described by Bhalla et al., Brody et al., Helbich et al.,and Santamaria et al. Efficacy of the scoring system with respect to PFT was compared.
  
  
  METHODS
  Inter-observer reliability of the scoring systems was tested using intraclass correlation (ICC) between the two observers. Spearman correlation coefficients were calculated between the scoring systems and between the scoring systems and PFT results.
  
  
  RESULTS
  In our study, right upper and middle lobes were the most frequently involved sites of involvement. Bronchiectasis and peribronchial thickening were the most frequent imaging findings. Scores with all four scoring systems were reproducible, with good ICC coefficient of 0.69. There was good agreement between senior radiologists in all scoring systems.
  
  
  CONCLUSIONS
  We noted pulmonary imaging abnormalities in a large majority (96%) of our CF patients. There was no significant difference in the CT scores observed from various systems. The CT evaluation system by Broody is detailed and time consuming, and is ideal for research and academic setup. On the other hand, the systems by Bhalla and Santamaria are easy to use, quick, and equally informative. We found the scoring system by Santamaria preferable over that of Bhalla by virtue of additional points of evaluation and ease of use, and therefore better suited for busy clinical practice.
authors.affiliation: Department of Radiology, Narayana Health, Bangalore, Karnataka, India.
authors.forename: Venkatraman
authors.initials: V
authors.lastname: Bhat
authors.affiliation: Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Atiqa Abdul
authors.initials: AA
authors.lastname: Wahab
authors.affiliation: Department of Radiology, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Kailash C
authors.initials: KC
authors.lastname: Garg
authors.affiliation: Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Janahi
authors.affiliation: Department of Biostastistics, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Rajvir
authors.initials: R
authors.lastname: Singh
keywords: CFTR I1234V mutation; HRCT; cystic fibrosis; high-resolution computed tomography; low-dose CT