High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening

OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe...

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Main Authors: Renata Wrobel Folescu Cohen, Tânia Wrobel Folescu, Marcia Cristina Bastos Boechat, Vania Matos Fonseca, Elizabeth Andrade Marques, Robson Souza Leão
Format: Article
Language:English
Published: Elsevier España 2019-10-01
Series:Clinics
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322019000100288&tlng=en
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author Renata Wrobel Folescu Cohen
Tânia Wrobel Folescu
Marcia Cristina Bastos Boechat
Vania Matos Fonseca
Elizabeth Andrade Marques
Robson Souza Leão
author_facet Renata Wrobel Folescu Cohen
Tânia Wrobel Folescu
Marcia Cristina Bastos Boechat
Vania Matos Fonseca
Elizabeth Andrade Marques
Robson Souza Leão
author_sort Renata Wrobel Folescu Cohen
collection DOAJ
description OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.
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spelling doaj.art-a17ceb8a42374ec4b289e5682f8b767e2022-12-22T02:40:17ZengElsevier EspañaClinics1980-53222019-10-017410.6061/clinics/2019/e1399High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screeningRenata Wrobel Folescu Cohenhttps://orcid.org/0000-0003-0596-4821Tânia Wrobel Folescuhttps://orcid.org/0000-0002-2958-5271Marcia Cristina Bastos Boechathttps://orcid.org/0000-0002-8128-5073Vania Matos Fonsecahttps://orcid.org/0000-0002-5452-7081Elizabeth Andrade Marqueshttps://orcid.org/0000-0003-3341-101XRobson Souza Leãohttps://orcid.org/0000-0003-0636-1520OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322019000100288&tlng=enLung DiseasesCystic FibrosisNeonatal ScreeningTomographyMicrobiology
spellingShingle Renata Wrobel Folescu Cohen
Tânia Wrobel Folescu
Marcia Cristina Bastos Boechat
Vania Matos Fonseca
Elizabeth Andrade Marques
Robson Souza Leão
High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
Clinics
Lung Diseases
Cystic Fibrosis
Neonatal Screening
Tomography
Microbiology
title High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
title_full High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
title_fullStr High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
title_full_unstemmed High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
title_short High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
title_sort high resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening
topic Lung Diseases
Cystic Fibrosis
Neonatal Screening
Tomography
Microbiology
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322019000100288&tlng=en
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