{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T17:55:35Z","timestamp":1777312535741,"version":"3.51.4"},"reference-count":50,"publisher":"Hamad bin Khalifa University Press (HBKU Press)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,4,9]]},"abstract":"<jats:p>\n                    <jats:bold>Background:<\/jats:bold>\n                    Computed tomography (CT) features of cystic fibrosis (CF) lung disease can be objectively quantified using current CT scoring systems to assess the extent and severity of the disease. The aims of this study were to calculate the Santamaria CT scores in adult patients with CF with the predominant CFTR I1234V genotype, determine its reliability, and correlate these parameters with lung function, microbial colonization, compliance to treatment, and exacerbations.\n                    <jats:bold>Methodology:<\/jats:bold>\n                    This retrospective observational study was conducted on adult patients with CF who were regularly followed up in the adult CF service at Qatar via CT scans that were taken not during an acute exacerbation. CT scans were scored using the Santamaria scoring system. Corresponding spirometry, microbiological data of sputum culture, and relevant clinical data were correlated with individual CT scores.\n                    <jats:bold>Results:<\/jats:bold>\n                    Only 23 of the 31 patients underwent CT when not in an acute exacerbation and were included in the study analysis. A total of 20 (87%) patients had the I1234V genotype. There was good agreement between the two radiologists on the Santamaria CT scores with an intraclass correlation coefficient (ICC) value of 0.991. Bronchiectasis was the most consistent finding, followed by interlobular and intralobular septal thickening. Patients with poor lung function and frequent exacerbations had significantly higher CT scores (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.015). The CT scores of patients colonized with\n                    <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                    were higher but nonsignificant (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.20). The mean CT scores were significantly higher in patients who were noncompliant to regular treatment than in those who were compliant (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u00a00.012).\n                    <jats:bold>Conclusion:<\/jats:bold>\n                    Santamaria CT scores comprise a reliable scoring system for adult patients with CF and can be used to determine the extent and severity of lung disease.\n                    <jats:italic>P. aeruginosa<\/jats:italic>\n                    colonization causes more structural lung damage than other common colonizing organisms. Noncompliance to treatment has a significant impact on the increasing severity of CF lung disease.\n                  <\/jats:p>","DOI":"10.5339\/qmj.2020.4","type":"journal-article","created":{"date-parts":[[2020,4,17]],"date-time":"2020-04-17T01:30:53Z","timestamp":1587087053000},"source":"Crossref","is-referenced-by-count":3,"title":["CT score and correlation with lung function and microbiology of adult patients with cystic fibrosis with predominant I1234V genotype in Qatar"],"prefix":"10.5339","volume":"2020","author":[{"given":"Merlin","family":"Thomas","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mehak","family":"Raja","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mutaz","family":"Albakri","sequence":"additional","affiliation":[{"name":"Department of Chest, Hamad General Hospital, Doha, Qatar"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mostafa","family":"Najim","sequence":"additional","affiliation":[{"name":"Department of Medicine, Hamad General Hospital, Doha, Qatar"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Prem","family":"Chandra","sequence":"additional","affiliation":[{"name":"Medical Research Centre Hamad Medical Corporation, Doha, Qatar"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mona","family":"Allangawi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3277","reference":[{"issue":"10","key":"CIT0001","series-title":"Am J Respir Crit Care Med.","first-page":"957","article-title":"Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health","volume":"176","author":"Flume\n\u00a0\nPA","year":"2007"},{"issue":"1","key":"CIT0002","series-title":"Clin Infect Dis.","first-page":"158","article-title":"Microbiology of sputum from patients at cystic fibrosis centers in the United States","volume":"27","author":"Burns\n\u00a0\nJL","year":"1998"},{"issue":"6","key":"CIT0003","series-title":"Curr Opin Pulm Med.","first-page":"492","article-title":"Pseudomonas acquisition in young patients with cystic fibrosis: pathophysiology, diagnosis, and management","volume":"9","author":"Rosenfeld\n\u00a0\nM","year":"2003"},{"issue":"1","key":"CIT0004","series-title":"Annals ATS.","first-page":"64","article-title":"Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis","volume":"12","author":"Danders\n\u00a0\nDB","year":"2015"},{"issue":"3","key":"CIT0005","series-title":"Radiology.","first-page":"783","article-title":"Cystic fibrosis: scoring system with thin-section CT","volume":"179","author":"Bhalla\n\u00a0\nM","year":"1991"},{"issue":"10","key":"CIT0006","series-title":"Pediatr Radiol.","first-page":"731","article-title":"High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate","volume":"29","author":"Brody\n\u00a0\nAS","year":"1999"},{"issue":"5","key":"CIT0007","series-title":"Pediatrics.","first-page":"908","article-title":"Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained?","volume":"101","author":"Santamaria\n\u00a0\nF","year":"1998"},{"key":"CIT0008","series-title":"Radiology.","first-page":"434","article-title":"Pulmonary disease assessment in cystic fibrosis: Comparison of CT scoring systems and value of bronchial and arterial dimension measurements","volume":"231","author":"de Jong\n\u00a0\nPA","year":"2004"},{"issue":"1","key":"CIT0009","series-title":"Indian J Radiol Imaging.","first-page":"44","article-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","volume":"25","author":"Bhat\n\u00a0\nV","year":"2015"},{"issue":"3","key":"CIT0010","series-title":"Ann Trop Paediatr.","first-page":"203","article-title":"Cystic fibrosis in a large kindred family in Qatar","volume":"20","author":"Abdul Wahab\n\u00a0\nA","year":"2000"},{"issue":"3","key":"CIT0011","series-title":"Am J Respir Crit Care Med.","first-page":"1107","article-title":"Standardization of spirometry, 1994 update","volume":"152","year":"1995"},{"key":"CIT0012","unstructured":"UK Cystic Fibrosis Registry Annual Data Report 2012\n."},{"issue":"1","key":"CIT0013","series-title":"Thorax.","first-page":"80","article-title":"Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis","volume":"61","author":"de Jong\n\u00a0\nPA","year":"2006"},{"issue":"3","key":"CIT0014","series-title":"Eur Respir J","first-page":"844","article-title":"Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?","volume":"42","author":"Loeve\n\u00a0\nM","year":"2013"},{"issue":"2","key":"CIT0015","series-title":"Radiology","first-page":"537","article-title":"Cystic fibrosis: CT assessment of lung involvement in children and adults","volume":"213","author":"Helbich\n\u00a0\nTH","year":"1999"},{"issue":"2","key":"CIT0016","series-title":"J Pediatr","first-page":"154","article-title":"Early morphologic changes in the lungs of asymptomatic infants and young children with cystic fibrosis","volume":"144","author":"Brody\n\u00a0\nAS","year":"2004"},{"issue":"1","key":"CIT0017","series-title":"J Pediatr","first-page":"32","article-title":"High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests","volume":"145","author":"Brody\n\u00a0\nAS","year":"2004"},{"issue":"1","key":"CIT0018","series-title":"Eur Respir J","first-page":"93","article-title":"Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis","volume":"23","author":"de-Jong\n\u00a0\nPA","year":"2004"},{"key":"CIT0019","series-title":"Pediatr Pulmonol","first-page":"393","article-title":"Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis","volume":"46","author":"Sanders\n\u00a0\nDB","year":"2011"},{"issue":"2","key":"CIT0020","series-title":"Radiology","first-page":"534","article-title":"Association between mucoid Pseudomonas infection and bronchiectasis in children with cystic fibrosis","volume":"252","author":"Farrell\n\u00a0\nPM","year":"2009"},{"issue":"1","key":"CIT0021","series-title":"J Bras Pneumol","first-page":"41","article-title":"High-resolution computed tomography scores in cystic fibrosis patients colonized with Pseudomonas aeruginosa or Staphylococcus aureus","volume":"38","author":"Folescu\n\u00a0\nTW","year":"2012"},{"issue":"2","key":"CIT0022","series-title":"Pediatr Pulmonol","first-page":"88","article-title":"Placebo-controlled, double-blind, randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonization in cystic fibrosis","volume":"25","author":"Wiesemann\n\u00a0\nHG","year":"1998"},{"issue":"3","key":"CIT0023","series-title":"Ther Adv Chronic Dis","first-page":"170","article-title":"Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications","volume":"7","author":"Edmondson\n\u00a0\nC","year":"2016"},{"issue":"6","key":"CIT0024","series-title":"Curr Opin Pulm Med","first-page":"687","article-title":"The impact of medication adherence on lung health outcomes in cystic fibrosis","volume":"19","author":"Eakin\n\u00a0\nMN","year":"2013"},{"issue":"5","key":"CIT0025","series-title":"J Cyst Fibrosis","first-page":"359","article-title":"Treatment compliance in children and adults with cystic 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exacerbations in children with cystic fibrosis","volume":"12","author":"Danders\n\u00a0\nDB","year":"2015"},{"issue":"3","key":"CIT0005","series-title":"Radiology.","first-page":"783","article-title":"Cystic fibrosis: scoring system with thin-section CT","volume":"179","author":"Bhalla\n\u00a0\nM","year":"1991"},{"issue":"10","key":"CIT0006","series-title":"Pediatr Radiol.","first-page":"731","article-title":"High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate","volume":"29","author":"Brody\n\u00a0\nAS","year":"1999"},{"issue":"5","key":"CIT0007","series-title":"Pediatrics.","first-page":"908","article-title":"Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained?","volume":"101","author":"Santamaria\n\u00a0\nF","year":"1998"},{"key":"CIT0008","series-title":"Radiology.","first-page":"434","article-title":"Pulmonary disease assessment in cystic fibrosis: Comparison of CT scoring systems and value of bronchial and arterial dimension measurements","volume":"231","author":"de Jong\n\u00a0\nPA","year":"2004"},{"issue":"1","key":"CIT0009","series-title":"Indian J Radiol Imaging.","first-page":"44","article-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","volume":"25","author":"Bhat\n\u00a0\nV","year":"2015"},{"issue":"3","key":"CIT0010","series-title":"Ann Trop Paediatr.","first-page":"203","article-title":"Cystic fibrosis in a large kindred family in Qatar","volume":"20","author":"Abdul Wahab\n\u00a0\nA","year":"2000"},{"issue":"3","key":"CIT0011","series-title":"Am J Respir Crit Care Med.","first-page":"1107","article-title":"Standardization of spirometry, 1994 update","volume":"152","year":"1995"},{"key":"CIT0012","unstructured":"UK Cystic Fibrosis Registry Annual Data Report 2012\n."},{"issue":"1","key":"CIT0013","series-title":"Thorax.","first-page":"80","article-title":"Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis","volume":"61","author":"de Jong\n\u00a0\nPA","year":"2006"},{"issue":"3","key":"CIT0014","series-title":"Eur Respir J","first-page":"844","article-title":"Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?","volume":"42","author":"Loeve\n\u00a0\nM","year":"2013"},{"issue":"2","key":"CIT0015","series-title":"Radiology","first-page":"537","article-title":"Cystic fibrosis: CT assessment of lung involvement in children and adults","volume":"213","author":"Helbich\n\u00a0\nTH","year":"1999"},{"issue":"2","key":"CIT0016","series-title":"J Pediatr","first-page":"154","article-title":"Early morphologic changes in the lungs of asymptomatic infants and young children with cystic fibrosis","volume":"144","author":"Brody\n\u00a0\nAS","year":"2004"},{"issue":"1","key":"CIT0017","series-title":"J Pediatr","first-page":"32","article-title":"High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests","volume":"145","author":"Brody\n\u00a0\nAS","year":"2004"},{"issue":"1","key":"CIT0018","series-title":"Eur Respir J","first-page":"93","article-title":"Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis","volume":"23","author":"de-Jong\n\u00a0\nPA","year":"2004"},{"key":"CIT0019","series-title":"Pediatr Pulmonol","first-page":"393","article-title":"Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis","volume":"46","author":"Sanders\n\u00a0\nDB","year":"2011"},{"issue":"2","key":"CIT0020","series-title":"Radiology","first-page":"534","article-title":"Association between mucoid Pseudomonas infection and bronchiectasis in children with cystic fibrosis","volume":"252","author":"Farrell\n\u00a0\nPM","year":"2009"},{"issue":"1","key":"CIT0021","series-title":"J Bras Pneumol","first-page":"41","article-title":"High-resolution computed tomography scores in cystic fibrosis patients colonized with Pseudomonas aeruginosa or Staphylococcus aureus","volume":"38","author":"Folescu\n\u00a0\nTW","year":"2012"},{"issue":"2","key":"CIT0022","series-title":"Pediatr Pulmonol","first-page":"88","article-title":"Placebo-controlled, double-blind, randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonization in cystic fibrosis","volume":"25","author":"Wiesemann\n\u00a0\nHG","year":"1998"},{"issue":"3","key":"CIT0023","series-title":"Ther Adv Chronic Dis","first-page":"170","article-title":"Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications","volume":"7","author":"Edmondson\n\u00a0\nC","year":"2016"},{"issue":"6","key":"CIT0024","series-title":"Curr Opin Pulm Med","first-page":"687","article-title":"The impact of medication adherence on lung health outcomes in cystic fibrosis","volume":"19","author":"Eakin\n\u00a0\nMN","year":"2013"},{"issue":"5","key":"CIT0025","series-title":"J Cyst Fibrosis","first-page":"359","article-title":"Treatment compliance in children and adults with cystic fibrosis","volume":"7","author":"Arias\n\u00a0\nLRP","year":"2008"}],"container-title":["Qatar Medical 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