scholarly journals RADIOLOGIC DIAGNOSTIC'S CAPABILITIES OF LUNG INJURY IN ADULT PATIENTS WITH CYSTIC FIBROSIS

2019 ◽  
Vol 15 (1-2) ◽  
pp. 51-58
Author(s):  
N.L. Morozova ◽  
M.M. Tkachenko ◽  
A.A. Romanenko
Keyword(s):  
Cystic Fibrosis ◽  
Lung Injury ◽  
Structural Changes ◽  
Diagnostic Value ◽  
Bronchial Tree ◽  
Adult Patients ◽  
Lung Damage ◽  
Ct Scans ◽  
Diagnostic Process ◽  
Adequate Treatment

Relevance. The viability of patients is determined by the degree of lung injury despite the fact that cystic fibrosis (CF) is a multiple organ disease. Early diagnosis and adequate treatment complex of cystic fibrosis prolong the lives of patients and improves its quality. Therefore, timely and detailed diagnosis of cystic fibrosis lung is particularly important problem. The central role for its solution belongs to radiological studies.     Objective. To refine the radiology’s capabilities in detecting lung changes in case of cystic fibrosis in adult patients and to determine the role of the computed tomography (CT) in the diagnostic process. Materials and methods. The results of radiography and CT scans of 15 patients, for whom the diagnosis of CF was established in adulthood, were analyzed. Results. The most typical radiographic and CT signs of lung damage were determined in adult CF patients. It has been shown that the radiographic changes in the lungs are non-specific for CF patients and are determined by the secondary inflammatory process. The diagnostic capabilities of CT scans in detecting subtle structural changes in the lung tissue and bronchial tree are underlined, which allows to diagnose light and atypical forms of CF in adults, determinate the stage and activity of the pathological process and the effectiveness of the treatment. Findings. The radiation studies were able to define the CF’s specific symptoms of lung lesions. Radiography of the thoracic cavity’s organs is a necessary step in the preliminary diagnosis for adult patients with CF or in the medical emergency's cases. The CT has the greatest diagnostic value in detecting subtle structural and functional specific changes of CF. The regular monitoring using CT scans is necessary for improving the control of the respiratory organs’ state and determine the further tactics for the particular patient.

Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis

2021 ◽  
pp. 2101344
Author(s):  
Alienor Campredon ◽  
Enzo Battistella ◽  
Clémence Martin ◽  
Isabelle Durieu ◽  
Laurent Mely ◽  
...  
Keyword(s):  
Machine Learning ◽  
Cystic Fibrosis ◽  
Ct Scan ◽  
Structural Changes ◽  
Clinical Status ◽  
Chest Ct ◽  
Ct Scans ◽  
Wall Thickening ◽  
Unsupervised Machine Learning ◽  
One Year

ObjectivesLumacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). This study aimed to assess lung structural changes after one year of lumacaftor-ivacaftor treatment, and to use unsupervised machine learning to identify morphological phenotypes of lung disease that are associated with response to lumacaftor-ivacaftor.MethodsAdolescents and adults with CF from the French multicenter real-world prospective observational study evaluating the first year of treatment with lumacaftor-ivacaftor were included if they had pretherapeutic and follow-up chest computed tomography (CT)-scans available. CT scans were visually scored using a modified Bhalla score. A k-mean clustering method was performed based on 120 radiomics features extracted from unenhanced pretherapeutic chest CT scans.ResultsA total of 283 patients were included. The Bhalla score significantly decreased after 1 year of lumacaftor-ivacaftor (−1.40±1.53 points compared with pretherapeutic CT; p<0.001). This finding was related to a significant decrease in mucus plugging (−0.35±0.62 points; p<0.001), bronchial wall thickening (−0.24±0.52 points; p<0.001) and parenchymal consolidations (−0.23±0.51 points; p<0.001). Cluster analysis identified 3 morphological clusters. Patients from cluster C were more likely to experience an increase in percent predicted forced expiratory volume in 1 sec (ppFEV1) ≥5 under lumacaftor–ivacaftor than those in the other clusters (54% of responders versus 32% and 33%; p=0.01).ConclusionOne year treatment with lumacaftor-ivacaftor was associated with a significant visual improvement of bronchial disease on chest CT. Radiomics features on pretherapeutic CT scan may help in predicting lung function response under lumacaftor-ivacaftor.

scholarly journals Diagnostic value of lung ultrasound versus chest CT in COVID-19

2020 ◽  
Vol 11 (2) ◽  
pp. 5-18
Author(s):  
S. S. Petrikov ◽  
I. E. Popova ◽  
V. M. Abuchina ◽  
R. Sh. Muslimov ◽  
L. T. Khamidova ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Lung Diseases ◽  
Structural Changes ◽  
Lung Ultrasound ◽  
Ground Glass Opacity ◽  
Diagnostic Value ◽  
Chest Ct ◽  
Ct Scans ◽  
Chest Computed Tomography ◽  
Ct Data

Lung ultrasound demonstrates a high diagnostic value in the assessment of lung diseases.Aim. To determine the diagnostic accuracy of lung ultrasound compared to chest computed tomography (CT) in the diagnosis of lung changes in COVID-19. Materials and methods. The retrospective study included 45 patients (28 men) aged 37 to 90 years who underwent polypositional lung ultrasound with an assessment of 14 zones. The study compared lung echograms with chest CT data in assessing the prevalence of the process and the nature of structural changes. The diagnostic accuracy, sensitivity, and specificity of lung ultrasound in comparison with CT scans were determined, 95% confidence intervals (CI) were calculated.Results. In 44 patients (98%), CT revealed pathological changes with subpleural localization in both lungs. Of these, in 30 cases, the inflammation was limited only to the subpleural parts, and in 14 cases, the changes spread to the basal parts of the lungs, while ultrasound revealed changes at the depth of the lesion no more than 4 cm. The lesion of 10–11 zones according to lung ultrasound corresponds to CT 1–2 degrees, the lesion of 13–14 zones — CT 3–4 degrees. The sensitivity of ultrasound to detect lung changes of various types was ≥ 92%. The highest sensitivity of 97.9% (95% CI: 92.8–99.8%) was determined for small consolidations on the background of interstitial changes (degree 1A+, 1B+), which corresponded to “crazy-paving” pattern on CT. The specificity depended on the nature of the changes and varied from 46.7 to 70.0%. Diagnostic accuracy was ≥ 81%, the maximum values of 90.6% (95% CI: 85.6–94.2%) were obtained for moderate interstitial changes (grade 1A) corresponding to ground-glass opacity (type one) according to CT data.Conclusion. The sensitivity of ultrasound to detect lung changes in COVID-19 is more than 90%. Lung ultrasound has some limitations: inability to determine the prevalence of the process clearly and identify centrally located areas of changes in the lung tissue.

P104 Multiple-breath nitrogen washout test and structural lung damage in adult patients with cystic fibrosis

2021 ◽  
Vol 20 ◽  
pp. S71
Author(s):  
A. Cherniak ◽  
S. Krasovskiy ◽  
Z. Naumenko ◽  
G. Nekludova ◽  
K. Mikhailichenko ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Adult Patients ◽  
Lung Damage ◽  
Nitrogen Washout

scholarly journals Nitrogen leaching in multiple breathing and structural changes in the bronchopulmonary system in adult patients with cystic fibrosis

2020 ◽  
Vol 30 (2) ◽  
pp. 193-203
Author(s):  
A. V. Chernyak ◽  
G. V. Neklyudova ◽  
S. A. Krasovskiy ◽  
K. Yu. Mikhaylichenko ◽  
Zh. K. Naumenko ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Structural Damage ◽  
Structural Changes ◽  
Pulmonary Ventilation ◽  
Nitrogen Leaching ◽  
Forced Expiratory Volume ◽  
Adult Patients ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Lung Capacity

The aim of this work was to assess the functional and structural disorders of the bronchopulmonary system detected by high-resolution computed tomography (HRCT) in adult patients with cystic fibrosis (CF), and to determine the correlation between them.Methods. A cross-sectional study included patients with CF (n = 54: 20 male and 34 female; median age 25 years) and healthy volunteers (n = 32: 12 male, 20 female; median age 25 years). A complex study of the pulmonary function test (PFT) was carried out; it included spirometry, bodyplethysmography and measurement of the diffusing lung capacity for carbon monoxide (DLCO), nitrogen leaching in multiple respiration (NLMR) and HRCT. Dyspnea was assessed using the Modified Research Council Scale (mMRC). NLMR measurements were performed using the Easy-One Pro module, MBW (NDD Medizintechnik AG, Switzerland). Analysis of HRCT data according to the Bhalla classification was performed by two independent, experienced radiologists.Results. The mean value (± SD) of the forced expiratory volume for 1 second (FEV1) was 63 ± 26% of the proper value, forced vital capacity of the lungs (FVC) was 86 ± 20% of the proper value, the ratio of FEV1/FVC was 61 ± 15%; residual lung volume (RLV) was 220 ± 71% of the proper value, the ratio of RLV/ total lung capacity (TLC) was 48 ± 13%, intrathoracic gas volume was 150 ± 33% of the proper value, DLCO was 80 ± 16% of the proper value, lung clearance index (LCI) was 16.9 ± 5.0; moment ratio (MR2) was 54.7 ± 34.1. Bronchoectases with predominant lesions of > 9 segments and bronchial lesions from V generation and more distal were found in all patients. Peribronchial infiltration and mucoid plugs were also diagnosed in almost all patients (94 and 96%, respectively), while bronchogenic cysts or abscesses, atelectasis/consolidation, bullae or emphysema were rarely detected (in 30, 35, 20, and 17% of cases, respectively). The parameters of NLMR were statistically significantly correlated with both the PFT parameters and the HRTC data.Conclusion. In adult patients with CF, there is a significant unevenness of pulmonary ventilation, progressing as structural damage to the bronchopulmonary system increases and the PFT worsens. With a statistically significant increase in MR, the involvement of not only the central, but also peripheral airways in the pathological process is emphasized. It has been established that in adult CF patients there is a strong correlation between LCI and the severity of structural changes, detected by HRCT, comparable in strength and significance to FEV1. 

scholarly journals Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and Cystic Fibrosis

2021 ◽  
Vol 10 (16) ◽  
pp. 3478
Author(s):  
Frederic Schlemmer ◽  
Agnes Hamzaoui ◽  
Sonia Zebachi ◽  
Aurelie Le Thuaut ◽  
Gilles Mangiapan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Clinical Score ◽  
Adult Patients ◽  
Operating Characteristics ◽  
Diagnostic Score ◽  
Receiver Operating Characteristics Curve ◽  
Diagnostic Work ◽  
Work Up ◽  
Ciliary Dyskinesia

Background: etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management. Methods: diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort. Results: among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10–35.99); p = 0.003), chronic ENT symptoms or diagnosed sinusitis (10.53 (1.26–87.57); p = 0.03), digestive symptoms or situs inversus (5.10 (1.23–21.14); p = 0.025), and Pseudomonas. aeruginosa and/or Staphylococcus aureus isolated from sputum (11.13 (1.34–92.21); p = 0.02) are associated with CF or PCD. Receiver operating characteristics curve analysis, using a validation group of 167 patients with bronchiectasis, confirmed the score’s performance with AUC 0.92 (95% CI: 0.84–0.98). Conclusions: a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.

scholarly journals P096 Long-term follow-up of adolescent and adult patients with cystic fibrosis: a single centre's experience

2021 ◽  
Vol 20 ◽  
pp. S68
Author(s):  
A.I. Yilmaz ◽  
G. Ünal ◽  
B.S. Kibar ◽  
P. Sevgi ◽  
O. Eĝil ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Adult Patients ◽  
Long Term Follow Up

scholarly journals 205 Folate deficiency in adult patients at a regional cystic fibrosis (CF) centre

2014 ◽  
Vol 13 ◽  
pp. S99
Author(s):  
L. Cassidy ◽  
A. Morton ◽  
C. Etherington ◽  
D. Peckham
Keyword(s):  
Cystic Fibrosis ◽  
Folate Deficiency ◽  
Adult Patients

Paranasal Sinus Development in Chronic Sinusitis, Cystic Fibrosis, and Normal Comparison Population: A Computerized Tomography Correlation Study

1997 ◽  
Vol 11 (4) ◽  
pp. 275-282 ◽  
Author(s):  
Hung Jeff Kim ◽  
Ellen M. Friedman ◽  
Marcelle Sulek ◽  
Newton O. Duncan ◽  
Charles McCluggage
Keyword(s):  
Cystic Fibrosis ◽  
Maxillary Sinus ◽  
Paranasal Sinus ◽  
Chronic Sinusitis ◽  
Correlation Study ◽  
Uncinate Process ◽  
Ct Scans ◽  
Lateral Nasal Wall ◽  
Transverse Diameter ◽  
Sinus Disease

Chronic sinus disease in patients with and without cystic fibrosis may have an impact on the pattern of paranasal sinus pneumatization. Arrest of pneumatization has been reported in both of these conditions. To assess the development of the paranasal sinuses in relationship to chronic sinusitis and cystic fibrosis (CF), a retrospective review of coronal CT scans of the age-matched patients with no previous sinus disease, patients with chronic sinusitis, and cystic fibrosis patients was conducted. The patients’ ages ranged from 4 to 17 years. The maxillary sinus volume, anteroposterior diameter, and greatest transverse diameter and height were determined using image analysis software after the coronal CT scans were scanned into Macintosh computer. The size of the maxillary sinus increased with advancing age in the control and chronic sinusitis group, but not in the patients with cystic fibrosis. The patients with cystic fibrosis had a statistically significant smaller maxillary sinus size. Approximately 50% of the patients with chronic sinusitis had anatomic anomalies, the most common being paradoxical middle turbinates. The CT scans of CF patients were characterized by uncinate process demineralization and medial displacement of the lateral nasal wall in the middle meatus, and decreased maxillary sinus pneumatization.

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