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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2015; 25(01): 44-51
DOI: 10.4103/0971-3026.150144
Chest Radiology

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

Venkatraman Bhat
Department of Radiology, Narayana Health, Bangalore, Karnataka, India
,
Atiqa Abdul Wahab
Department of Pediatrics, Hamad Medical Corporation, Doha, India
,
Kailash C Garg
Department of Radiology, Hamad Medical Corporation, Doha, India
,
Ibrahim Janahi
Department of Pediatrics, Hamad Medical Corporation, Doha, India
,
Rajvir Singh
Department of Biostastistics, Hamad Medical Corporation, Doha, India
› Author Affiliations Financial support and sponsorship Nil.
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Abstract

Background and Aims: 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. Materials and 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. Statistical Analysis: 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. Conclusion: 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.

Keywords

CFTR I1234V mutation - cystic fibrosis - high-resolution computed tomography - HRCT - low-dose CT


Publication History

Article published online:
30 July 2021

© 2015. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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